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 Table of Contents  
POSTER ABSTRACT
Year : 2022  |  Volume : 17  |  Issue : 5  |  Page : 25-203

Posters Abstracts


Date of Web Publication26-Nov-2022

Correspondence Address:
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-3698.362016

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How to cite this article:
,. Posters Abstracts. Indian J Rheumatol 2022;17, Suppl S1:25-203

How to cite this URL:
,. Posters Abstracts. Indian J Rheumatol [serial online] 2022 [cited 2023 Feb 1];17, Suppl S1:25-203. Available from: https://www.indianjrheumatol.com/text.asp?2022/17/5/25/362016




  POS001 Top


Myofibroblast and pro-fibrotic cytokines in fibrosis of IgG4-related disease patients from South Asia: Preliminary data

Josna Joseph, V Prabhu, Meera Thomas1, K Rekha2, Thomas Alex1, John Mathew*; Departments of Clinical Immunology and Rheumatology, 1Pathology and 2Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India

Background: Myofibroblasts are specialized, activated fibroblasts found generally in fibrotic tissue and has excessive collagen secretion and contractile properties. There exists a paucity of data on the mechanistic role of pro-fibrotic cytokines in Indian IgG4-RD patients. Efficient treatment strategies could be devised, if the exact pathomechanism of myofibroblast activation in IgG4-RD associated fibrosis could be known.

Objective: To analyse ambispectively the presence of myofibroblasts and pro-fibrotic cytokines, IFN gamma and IL-33 involved in IgG4-RD associated fibrosis by immunohistochemical staining of biopsy samples from South Asian patients.

Methods: Archived biopsy samples of definite/ probable/ possible cases of IgG4-RD, classified according to classification criteria (1), taken from patients who attended the OPD and IPD of our tertiary care centre during January 2015 – January 2020 (12 nos.) were selected for this study. The paraffin sections were examined qualitatively for fibrosis and the excessive collagen deposition by histological staining like Hematoxylin and Eosin, Masson's Trichrome. Also, semi-quantitatively for the presence of alpha-Smooth muscle actin (α-SMA) expressing myofibroblasts and the involvement of pro-fibrotic cytokines (IFN-gamma, IL-33) by Immunohistochemistry. The presence of myofibroblasts and fibrogenic cytokines detected was semi quantitatively scored (+mild, ++moderate, +++ severe) and analysed.

Results: In the biopsy tissue sections of classified cases of IgG4-RD, myofibroblasts were present in 10/12 patients, moderate levels in 4 (33%) and very high (+++) in 3(25%). When IFN-gamma expressed at low levels in 6 (50%) and absent in 6 (50%), all the patients showed IL-33 expression with very high levels in half of the samples (6, 50%).

Conclusion: Profibrotic cytokine, IL-33 shows upregulation in the biopsy samples of IgG4-RD patients, suggesting a plausible role in fibrosis regulation. Further studies are warranted to identify earlier markers of fibrosis onset.


  POS002 Top


Prevalence of LTBI in patients with autoimmune diseases and in general population and specificity of Interferon Gamma Response Assay

Yogitha Chennault, S Chandrashekara, P Renuka; ChanRe Rheumatology and Immunology Centre and Research, Bengaluru, Karnataka, India

Background and Aims: In India, there are limited information on the prevalence of LTBI in the Autoimmune rheumatic disease population. To estimate the prevalence of LTBI positivity by Interferon gamma response assay (IGRA) among the patients with ARD and to compare the predictability of Tuberculosis in patients exposed to biologicals.

Methods: A retrospective database study, 1064 patients who were tested with IGRA from 2019 to 2022, were collected. The primary diagnosis, IGRA results and the Tuberculosis relapse during the follow-up with biologic exposure were collected.

Results: Among 1064 patients were tested, 941 for pre-assessment to biologics and 121 to exclude TB and 2 were excluded from analysis. IGRA was positive in 125 (13.28%) biologic screening and 40 (33.06%) when tested for TB screening and 816 (86.72%) patients screened for biologics were negative and 81 (66.94%) were suspects. 9 (0.85%) patients, developed active TB out of which 6 were screened for biologics and 2 (33.3%) were positive and 1 received prophylaxis and 4 (66.7%) negative and received biologics. 3 out of 9 were suspected and have developed active TB in 2 (66.67%) with IGRA negative out of which 1 had past TB infection and 1 (33.33%) with IGRA positive had developed active TB. Specificity of the IGRA in patients receiving biologics is 97.25% and in suspects is 66.95%. [Table 1].

Conclusion: In patients who received biologics, 6 out of them have developed a flare out of which 4 (66.67%) were negative and 2 patients have developed flare despite prophylaxis out of which 1 had received prophylaxis after biologics in view of high disease activity. In suspected patients, 3 have developed active disease and out of which 1 had positive IGRA and had developed active disease despite prophylaxis and in 2 patients with IGRA negative, 1 had past TB infection.


  POS003 Top


Rheumatology training in India: What needs to be done: A consultant perspective

Divya Lala1,2, Kunal Patil3, Yojana Gokhle4; 1Department of Rheumatology, Reliance Hospital, 2Department of Rheumatology, Fortis Hospital, 4Department of Rheumatology, Lokmanya Tilak Municipal Medical College, Sion Hospital, Mumbai, 3Sanjivani Rheumatology Clinic, Dr D. Y. Patil Medical Collage, Kolhapur, Maharashtra, India

Background: Rheumatology is an emerging specialty of medicine in India. Rheumatology training needs to be structured and comprehensive. We surveyed rheumatology consultants regarding their perception and perspectives about rheumatology training in India.
Table 1: Descriptive statistics (n=1064)

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Objectives: This study was aimed to explore the unmet need for improvement in training and the changes that need to be introduced to improve the training.

Methods: An online questionnaire was circulated virtually to randomly chosen rheumatology consultant across India. Questionnaires had fixed responses as well as open end responses which was designed to assess duration of rheumatology training, interdepartmental rotation, procedure needs to be trained, development of structured referral system and structured programme of rehabilitation etc.

Results: The questionnaires were virtually sent to 80 consultants among which 52 responded (52/80). Mean duration practice experience was 10.4 (+/-6.2) most of being from private setup (40/52). 70% consultant underwent training in India.58 % responded that duration of training should be of 3 year.100% respondent felt that there should be rheumatology training in UG and PG training days. Other department rotation included in curriculum should be biochemistry (92%), radiology (92%) physiotherapy (82%) occupation therapy (52%). 84% respondent felt there should be exchange programme amongst Indian institution. 76 % respondent felt there need of training for structured programme for rehabilitation.

Conclusion: Rheumatology training in India can be improved by training during UG and PG curriculum. There should be interdepartmental rotation during rheumatology training. There should be uniform curriculum across India along with exchange programme amongst institution.


  POS004 Top


Oxidative stress and inflammation in healthy subjects residing in polluted zones of a metropolitan city in India: A cross-sectional study

Uma Kumar, Maumita Kanjilal, Maheswari Thangavelu, Lakshmy Ramakrishnan1; Departments of Rheumatology and 1Cardiac Biochemistry, All India Institute of Medical Sciences, New Delhi, India

Background: Air pollution is an important factor risk factor for oxidative stress and inflammation leading to immune response.

Objectives: To study the prevalence of oxidative stress and inflammation in healthy subjects residing in the most polluted zones of Delhi -NCR.

Methods: A cross-sectional study was conducted on healthy subjects between 18 to 60 years of age, residing in highly air polluted zones of Delhi NCR, India for the past 10 years and whose houses were within a one-kilometre radius distance from the air quality monitoring stations. AIIMS Human Ethics Committee approved the study. Pregnant and lactating females were excluded.

Results: 350 healthy subjects were recruited. The mean age of subjects was 37.09±9.65 years with male-to-female ratio being 1:1. The mean body mass index (BMI) was found to be 25.83 ± 5.25 kg/m2. The inflammatory markers IL-6, TNF-α, and Hs-CRP were elevated in 87% (306/350), 15% (54/350) and 17% (61/350) subjects respectively. Markers of oxidative stress were elevated in 76% of subjects; Malondialdehyde (MDA) in 68% (239/350) and total oxidant status (TOS) in 76% (266/350) while both were elevated in 54% (190/350) subjects. Antioxidant total antioxidant status (TAS), Glutathione (GPX1) and superoxide dismutase (SOD) were reduced by 66% (230/350), 9% (31/350) and 36% (127/350) subjects respectively. There was a positive correlation between IL-6 and TOS and a negative correlation between IL-6 and TAS. Correlation of IL-6 with markers of oxidative stress OSI shown in [Table 1].
Table 1: Spearman-rank correlation coefficient analysis of IL-6 with markers of oxidative stress

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Conclusion: 76% subjects residing in the selected polluted zones of Delhi, NCR had elevated levels of TOS while 87% participants had elevated inflammatory markers reinforcing the fact that oxidative stress and inflammation are interconnected. Individuals between the age group 18-40 years were more prone to oxidative stress due to their more outdoor activities.


  POS005 Top


To study correlation between seropositivity and disease severity in rheumatoid arthritis

K P Rohith, J Deepthy, M Gautam1, Ramakant1; Departments of General Medicine and 1Indian Navy Hospital Ship Asvini, Mumbai, Maharashtra, India

Background: The rheumatoid factor (RF) and anti- citrullinated protein antibodies (ACPA) blood test is the most commonly adopted test for the diagnosis of rheumatoid arthritis (RA). RA patients who are seropositive for RF and/or ACPA face a greater likelihood of developing more aggressive symptoms.

Objectives and Methods: Our goal was to study the demographic, clinical characteristics and disease severity, as well as their correlation with RF or/and ACPA seropositivity, among a series of 61 RA patients aged >18 years who attended rheumatology clinic at INHS Asvini, Mumbai.

Results: Of the 61 RA patients included in this study all were male. Demographic data: Mean age of population was 40.84 +/- 10.40 years, Habits: Smoking-17 (27.8%) and Alcohol-16 (26.2%), 23 patients had comorbidities. Average duration of RA was 4.13 years (SD: 3.97). Forty patients were seropositive (RF positive: 32, CCP positive: 22, both positive: 15) and 21 were seronegative [Table 1]. Twenty-two (36.1%) patients had abnormal findings on X-ray of hand at the time of evaluation. Subcutaneous was the most common extra articular manifestation seen in 7 (11.5%), one each had ILD and colitis, none had red eye/dry eye or dry mouth [Table 2]. Skeletal deformity was present in 13 patients. Mean with SD of ESR, CDAI and DAS 28 was -18.70 mm Hr (12.362), 11.549 (10.3335) and 4.467 (6.7795) respectively. cDMARDs: 93.4% patients were on methotrexate therapy, 37.7% and 9.8 % were on hydroxychloroquine and leflunomide therapy respectively. Almost 64% of patients were on steroid therapy. bDMARDs usage was seen in about 36% patients. No significant associations between seropositivity and disease severity was seen based on CDAI and DAS 28 scoring system (Coefficient: 0.204, p value: 0.17). However mean CDAI and DAS 28 score was higher in seronegative group. (10.038 vs. 14.429, 3.396 vs. 6.229).
Table 1: Clinical disease activity index and disease activity scores 28 scoring in seropositive and seronegative group

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Table 2: Deformity and extra articular

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Conclusion: RF and ACPA seropositivity and seronegativity status RA patients were found to have similar disease severity based on articular- extra articular manifestations, CDAI and DAS 28 scoring system. Both scoring systems had similar results in both groups.


  POS006 Top


Study on monitoring of methotrexate liver toxicity with ultrasound abdomen in rheumatoid arthritis: A retrospective observational study

Anjana G Varier, Hima Sreekumar, S R Lekshmi, Vishad Viswanath; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India

Background: Methotrexate has been the anchor drug for treatment of Rheumatoid Arthritis (RA) for decades. Hepatic fatty infiltration, fibrosis with a potential to progress to cirrhosis, is one of the most severe adverse effects of long-term methotrexate treatment. Concerns about hepatotoxicity has led to the implementation of guidelines for intensive monitoring, including the use of surveillance liver biopsy, imaging and liver function tests at regular intervals of methotrexate treatment. In this Study we look at the utility of screening Ultrasound Abdomen in monitoring of methotrexate liver toxicity.

Objective: To assess the Outcome of screening Ultrasound Abdomen in monitoring of liver toxicity in Rheumatoid Arthritis patients on Methotrexate.

Methods: This study is a retrospective Observational study of RA patients under follow-up in our institution. Seropositive RA patients with atleast one Ultrasound Abdomen done during treatment period were selected. The Outcome was assesed as Stopped, Tapered or continued methotrexate according to Ultrasound liver finding.

Results: 60 patients were studied. The mean age of patients were 52 years with mean duration of illness 10 years. Mean duration of treatment was 3 years.12 patients stopped methotrexate treatment based on USG finding out of which 2 patients had Grade I fatty liver with transaminitis, 1 patient had Grade II fatty liver with transaminitis, 4 patients had Grade II fatty liver without transaminitis, 1 patient had grade III fatty liver with transaminitis, 2 patients had cirrhosis with transaminitis and 2 patients had cirrhosis without transaminitis. 58% of patients who stopped methotrexate had cumulative dose less than 1500 mg of methotrexate.

Conclusion: Chronic liver disease can occur with normal liver function tests. Periodic screening of RA patients with Ultrasound abdomen during treatment could help in reducing risk of long term methotrexate liver toxicity.


  POS007 Top


Disease perceptions, anxiety and depression in patients with rheumatoid arthritis: A cross-sectional study

P M Ankush, Nishant G Kamble, Sayan Mukherjee, Abilash V Krishnan, Mukesh K Maurya, Urmila Dhakad; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Rheumatoid arthritis (RA) is the most common autoimmune arthritis worldwide. Treatment of RA includes pharmacotherapy and physical therapy. Research based on Leventhal et al's common-sense model of illness has shown that the way patients make sense of their disease can strongly influence their quality of life. Depression and anxiety are also associated with functional disability and pain perception of RA patients.

Objectives: To study Disease Perceptions and prevalence of Anxiety and Depression in patients with RA.

Methods: Patients diagnosed with RA as per ACR 2010 criteria, attending OPD were interviewed. Pregnant females, patients with infections, psychiatric disorder, CKD, Heart Failure, chronic lung and liver disease were excluded. Disease perception was measured using Brief Illness Perception Questionnaire (BIPQ) (Total Score 0-80). Anxiety and Depression were assessed using Hospital Anxiety and Depression Questionnaire (HADS).

Results: A total of 78 patients were interviewed. Mean age was 41.6 years and 89% of them were females. 35.8% patients had low disease activity or were in remission and 74.2% patients had moderate to high disease activity. 53.8% had either borderline or abnormal anxiety score and 51.2% patients had borderline or abnormal depression score. Mean total BIPQ was 45.05 and item 6 on BIPQ (patients concern) had highest mean score of 8.06, followed by item 8 (emotional impact) (i.e., 7.18). Mean score for item 4 (impact of treatment) was the lowest (2.36).

Conclusions: Half of our patients had borderline or abnormal anxiety and depression score. Patients were greatly concerned and emotionally disturbed due to the illness. On the positive side, their perception about benefit of treatment was really good. Poor perception, anxiety and depression contribute to poor quality of life, treatment adherence and functional disability. There is a dire need for interventions specifically targeting these issues.


  POS008 Top


Safety of tacrolimus in patients with rheumatoid arthritis associated interstitial lung disease

Sharath Kumar, Vikramraj K Jain, S Nagaraj, D Ranjitha, V A Deepika Ponnuru; Optima Super Speciality Hospitals, Bengaluru, Karnataka, India

Background: Tacrolimus has proven effective for rheumatoid arthritis and Myositis-ILD. However, knowledge of its utility in RA -ILD is restricted to only 2 publications from Japan. We analysed data of RA-ILD patients from our centre who were on tacrolimus to determine the safety and efficacy of the same.

Objectives: To determine safety of tacrolimus in patients with RA-ILD as well as outcomes (mortality, respiratory admission).

Methods: Retrospective review of RA-ILD patients from our centre treated with tacrolimus between January 2015 and July 2021. Patients demographic details, duration of ILD, any hospitalisations due to respiratory illness, worsening of serum creatinine, development of diabetes and HTN during the study period were determined. In addition, the requirement of oxygen and steroids at the last follow up visit and deaths were recorded and analysed.

Results: Twenty-four of 35 RA-ILD patients were treated with Tacrolimus. Patient characteristics are mentioned in [Table 1]. During cumulative follow up 85 patient-years none experienced worsening of serum creatinine. Diabetes and HTN was diagnosed de-novo in 3/15 (20%) and 7/15 (46.7%) patients respectively. Most patients were off supplemental oxygen at last follow-up (20/24). Half on daily steroids at last follow up, most on.
Table 1: Characteristics of rheumatoid arthritis associated interstitial lung disease patients treated with tacrolimus

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Conclusion: In this data set, tacrolimus seemed to be safe in RA-ILD with no cases of nephropathy. Development of HTN as well as diabetes due to the medication is a concern. Most patients at last follow-up had not had respiratory admissions, were alive and off supplemental oxygen. However, lack of follow up PFTs and lack of comparative group makes any inference of efficacy tenuous at best. Prospective randomized controlled studies are needed.


  POS009 Top


Therapeutic impact of curcumin on inflammation and endothelial dysfunction in adjuvant induced arthritic rats: CiRA study

Inderjeet Verma, Manni Rohilla, Ashit Syngle1, 2, 3, Devaansh Syngle3, Anmol Singh Bains3; M. M. College of Pharmacy, Maharishi Markandeshwar (Deemed to be University), Mullana-Ambala, Haryana, 1Healing Touch City Clinic, Chandigarh, 2Fortis Multi Specialty Hospital, Mohali, Punjab, 3Healing Touch Foundation, Chandigarh, India

Background: Cardiovascular disease is leading cause of death RA due to increased CV risk resulting from inflammation-induced endothelial dysfunction and accelerated atherosclerosis. The management of this enhanced CV risk in RA is still evolving.

Objective: To investigate the impact of Curcumin on endothelial function in Adjuvant Induced Arthritis animal.

Methods: Arthritis was induced in Wistar rats by complete Freund's adjuvant (FCA), and treated with curcumin (150 and 300 mg/kg/day) and methotrexate (7.5 mg/kg/week) after onset of arthritis (day 21) until day 42. The effects of curcumin were assessed by arthritis score, inflammatory serum cytokines, oxidative measures as well as histological changes in aorta of Wistar rats.

Results: Curcumin (150/300 mg/kg) had significant impact on arthritis score, paw thickness, ESR and CRP level in Wistar rat as compared to disesae control group while curcumin 300 mg/kg has shown almost similar to MTX treated group. Curcumin (300 mg/kg) also reduced pro-inflammatory cytokines – TNF-α, and IL-6, which levels were close to MTX treated group. We also found that both the doses of curcumin demonstrated substantial improved oxidative measures like superoxide dismutase (SOD) and glutathione (GSH) level as well as a significant decrease in nitric oxide (NO) levels compared with disease control. Interestingly, the level of NO, SOD and GSH was slightly more improved in curcumin 300 mg/kg group than the MTX treated group. The protective effect of curcumin on endothelial function was also evident from the histological changes in aorta [Figure 1] and its effect was found comparable to that of MTX treated animals.
Figure 1: Histopathological images of the aorta of Wistar rat in different experimental groups. Histopathology findings that normal control group. (a) The normal endothelial cell are present on the endothelial layer without any destruction in the endothelium layer. Disease control group. (b) Abnormality in the endothelial cell on the endothelial layer in this figure endothelial cells are less and become flat which are the sign of destruction in the endothelium. MTX treated group. (c) Minimum destruction in the endothelium and some some endothelial cells are flat VC treated group. (d) Also abnormality in the endothelial cells on the endothelium in which endothelial cells are became less and flat due to destruction on the layer CRM 150 mg/kg dose group. (e) The endothelial cells are normal along with some minimum flat cells. CRM 300 mg/kg dose group (f) The normal and more cells are present on the endothelium, there is no destruction in the cells and endothelium was measured as normal

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Conclusion: This is the first study to explore the impact of curcumin on endothelial dysfunction in RA. This current study demonstrated that AIA experimental rat's treatment with curcumin (150/300 mg/kg/day) has anti-inflammatory, antioxidant and vasculo-protective potential. Curcumin 300 mg/kg/day) has extremely similar therapeutic impact with MTX. However, further pharmacological investigations are warranted.


  POS010 Top


A molecular docking and simulation study targeting STAT-I through active phytocompounds of Curcuma caesia

Ankita Pati, Jyoti Ranjan Parida, Dattatreya Kar, Ananya Kuanar; Centre for Biotechnology, Siksha 'O' Anusandhan University, Bhubaneswar, Odisha, India

Rheumatoid Arthritis is a chronic, inflammatory, and systemic autoimmune disease, it affects people worldwide, where higher production of Signal transducer and activator of transcription 1 (STAT-I) is associated with this form of the disease. Traditionally used medicinal plants contain a large amount of bioactives and pave a new path to develop drugs and medications for Rheumatoid Arthritis. Curcuma caesia is an endangered plant endemic to North-East and Central India that has several medical applications such as bronchitis, dysentery, diarrhoea, tumours, cancer, and cosmetic applications as [Figure 1]. Curcuma caesia's biological and therapeutic activities have been attributed mostly to its bioactive ingredients such as camphor, curcuminoids, phenolics, flavonoids, protein, amino acids, essential oil, and alkaloids. In reality, the primary ingredient, camphor, curcuminoids, has been linked to a variety of pharmacological actions that can be used to treat arthritis and its accompanying inflammatory responses. The present study was aimed to examine the potential of Curcuma caesia bioactive compounds against STAT-I. Interferons (IFNs) and other cell signals are known to be the major reason behind the activation and production of human STAT-I by translocating to the nuclei and activating transcription of IFN-stimulated genes. In this study, 72 compounds from Curcuma caesia were taken based on traditional knowledge linkage with inflammatory-like disease treatment, were screened against the Homology-Modelled structure of STAT-1 with the objective of identifying some active phytochemicals as inhibitors. The entire study was carried out using AutoDock Vina 1.2 and different modules of Schrodinger Suite 2020-3. During the docking of the phytochemicals, a compound, Delta-Cadinene from Curcuma caesia showed the best binding affinity with the receptor with a Docking Score of −7.3. In order to study the binding stability, the complex between the STAT-1 and Delta-Cadinene was subjected to 100 ns Molecular Dynamics simulation using Desmond module of Schrodinger suite 2020-3, during which the receptor-ligand complex showed substantial stability after 50 ns of MD Simulation [Figure 2]. The phytocompound Delta-Cadinene also showed promising results during ADME-Tox and bioactivity score prediction analysis performed using Swiss ADME, pkCSM, ProTox II and Molinspiration servers, respectively. The compound Delta-Cadinene is suggested as a potential STAT-1 inhibitor based on the results of this analysis, which may then block the immunosuppressive mechanism in the human body during the start of the disease. Further research built on this study would pave the way for the accurate and long-lasting identification of a viable therapeutic regimen for the treatment and management of rheumatoid arthritis.
Figure 1: Medical applications

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Figure 2: Dynamic simulation

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  POS011 Top


Can we predict response to methotrexate in rheumatoid arthritis: Results from a multicentre, randomized controlled trial on methotrexate escalation in rheumatoid arthritis

Siddharth Jain, Varun Dhir, Amita Aggarwal1, Ranjan Gupta2, Bidylaxmi Leishangthem, Shankar Naidu, Aastha Khullar, Veena Dhawan3, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain; Department of Internal Medicine, Division of Clinical Immunology and Rheumatology, Postgraduate Institute of Medical Education and Research, 3Department of Experimental Medicine and Biotechnology, Postgraduate Institute of Medical Education and Research, Chandigarh, 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 2Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Background: Methotrexate (MTX) is the gold standard, first-line therapy for rheumatoid arthritis (RA). However, not all patients respond to MTX, and the predictors of its response or non-response have not yet been reliably identified. Identification of these predictors will facilitate personalized therapeutic choices, and improve patient outcomes.

Objectives: To identify clinico-laboratory predictors of response to MTX monotherapy in active RA.

Methods: This study included patients with active RA (SJC ≥2 and TJC ≥4) aged 18-55 years, with disease duration <5 years, who were not receiving DMARDs (except HCQ and low-dose prednisolone) and had been enrolled in the multicentre, parallel group RCT comparing two different MTX escalation strategies in RA (MEIRA). All these patients received MTX monotherapy which was started at 15 mg/week, escalated to 25 mg/week by 4-8 weeks, and continued till 16 weeks. MTX response was defined as EULAR good or moderate response (based on DAS28-3v) at 16 weeks. Stepwise, multivariable logistic regression was done using key demographic (age, gender, BMI, comorbidities), clinical (disease duration, DAS28, HAQ), and laboratory parameters (RF, anti-CCP, ESR, CRP, RBC MTX-polyglutamates, IL-6, MMP-3) as independent variables to identify predictors of MTX response. Two-tailed p-value <0.05 was considered statistically significant. (Trial Reg: CTRI/2018/12/016549).

Results: Out of a total of 178 included patients [84% females, mean age 40 (9) years, mean DAS28-CRP=5.4 (1.1)], 113 (63.5%) were classified as MTX responders at 16 weeks. Age (OR=0.95, p=0.01), BMI (OR=1.12, p=0.006), and RF (OR=0.34, p=0.045) were found to be independent predictors of MTX response on multivariate analysis [Table 1]. On sensitivity analysis with DAS28-ESR-based EULAR response, age (OR=0.94, p = 0.003) and RF (OR=0.42, p = 0.059) were replicated as independent predictors of MTX response, in addition to pre-treatment swollen joint count (OR=0.94, p = 0.05).
Table 1: Multivariable logistic regression analysis for prediction of methotrexate response (European league against rheumatism good or moderate response based on disease activity scores 28 -C-reactive protein) in rheumatoid arthritis

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Conclusion: Younger age, RF negativity, higher BMI, and lower pre-treatment swollen joint count are potential predictors of response to MTX monotherapy in RA.


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Self-assessment of joints in RA: Is it worth pressing for?

S Sri Lakshmi, Sandeep Kansurkar, Deepti Agarwal, Kavita Krishna; Bharati Vidyapeeth Medical College and Hospital, Pune, Maharashtra, India

Background: Self-assessment of joints by patients, if reliable and accurate can be used as a potential clinical tool in rheumatoid arthritis. Traditionally, joint examination is done by clinicians. However, patient self-joint count is explored in this study to incorporate the technique in day-to-day clinical practice. The utility of patient self-joint counts has become an increasingly important area to explore, with remote disease monitoring and telehealth taking on a larger role across rheumatology practices in this global pandemic. This study is inspired from the video released by Centre for Epidemiology and Arthritis on self-assessment of joints in patients with RA.

Objectives: This study aims to assess the joint involvement by patients and compare it with clinician in terms of accuracy to include the same in routine clinical practice as a diagnostic tool.

Methods: Consecutive rheumatoid arthritis patients over the age of 18 were included in this study. Total sample size was 43. Patients were given an instruction manual on how to examine the joints in English and regional language. If the patient had difficulty in reading, the attendant was allowed to explain the same to the patient. Then patient filled a form with pictorial representation of the joints and joints with swelling and pain was marked. They also scored the pain, difficulty of the questionnaire and patient global assessment score. This was followed by clinician assessment and physician global assessment.

Results: The total number of joints involved as counted by the clinician was 499 and by the patient was 559. The sensitivity was 86.02 % (82.6- 88.9%) and specificity was 95.62 % (94.8-96.3%) [Table 1]. Among the joint examination, the patient assessment of shoulder (Sp- 91.95%) and elbows (Sp-96.18%) was perceived better by the patient [Figure 1]. Whereas more distal joints like wrist, MCP, Knee and ankle was perceived more accurately by the clinician. There was high false positivity rate with MCP and PIP joints.
Figure 1: Sensitivity and specificity of joint examination by patient

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Table 1: Sensitivity and specificity of joint examination: Self-assessment of rheumatoid arthritis

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Conclusion: This study shows that self-assessment of joints by patient is a reliable tool in monitoring disease activity.


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Evaluation of myocardial function in patients with rheumatoid arthritis using strain ECHO and correlation with duration of disease and disease severity

Kavitha Mohanasundaram, G S Sanjay Surya, Sanjeeiv Krishnan, Gowtham Hanumanram, A Gowrishankar; Saveetha Medical College, Chennai, Tamil Nadu, India

Background: Rheumatoid arthritis (RA) is a systemic autoimmune disease, characterised by chronic inflammatory changes. Pro inflammatory cytokines released in RA has a negative impact of cardiac health. Individuals with RA have shorter life expectancy and their risk of cardiovascular death is more than 50 % higher than that of the rest of the population. Early myocardial dysfunction in RA patients may be detectable sooner using speckle-tracking echocardiography.

Materials and Methods: This cross sectional study was conducted in 65 patients with rheumatoid arthritis and 65 healthy controls (mean age = 36 years) at a tertiary health care centre. Two-dimensional and Doppler echocardiograms were performed on all subjects by a single ECHO operator. Strain analysis was done by speckle-tracking echocardiography. Statistical analyses was performed by using a statistical software package SPSS, version 20.0.

Results: The mean Global Longitudinal Strain values in rheumatoid arthritis patients was lesser than controls and it was statistically significant [p =0.001, [Table 1]]. Duration of RA when more than 5 years had significant impact on GLS and had reduced GLS when compared to subjects whose disease duration was less than 5 years of disease [Table 2]. Presence or absence of erosions did not change GLS. No statistical difference was noted when GLS was compared in patients with seropositive and seronegative rheumatoid arthritis.
Table 1: Comparison of demographic, acute-phase reactants and speckle-tracking echocardiography between case and control by Student's t-test

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Table 2: Comparison of global longitudinal strain with disease duration, erosions, rheumatoid factor and anti cyclic citrullinated peptides positivity in rheumatoid arthritis

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Conclusion: Patients with RA and no clinical Cardiovascular disease have reduced Left ventricular systolic function as shown by lower Global Longitudinal Strain. It is associated with disease activity and RA disease duration. Routine Speckle-tracking method can be employed regularly in RA patients for early detection and treatment.


  POS014 Top


Comparison of two schedules for administering oral methotrexate (split dose vs. single dose) once weekly in patients with active rheumatoid arthritis: An open label, parallel group, randomized controlled trial split-dose methotrexate in active rheumatoid arthritis trial

Chandra Bhushan Prasad, Varun Dhir, Bidyalaxmi Leishangthem, Aastha Khullar, G S R S N K Naidu, Priya Saini, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain; Department of Internal Medicine, Clinical Immunology and Rheumatology Unit, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Methotrexate (MTX) is a well-known anchor drug for rheumatoid arthritis (RA). Splitting the oral MTX (>15 mg per week) is an option to improve its bioavailability as well as efficacy.

Objective: To compare the efficacy, safety and tolerability of split-dose versus single dose once weekly regimen of oral MTX in patients with active RA.

Methods: SMART is ongoing 24-week multicentre, open-label (assessor blinded) RCT which proposes to enroll 250 patients age 18-60 years having active RA with disease duration <5 years. Here we present 16-week data from single centre. Patients were randomized 1:1 into either oral split-dose MTX (10 mg in morning and 15 mg in evening on same day once a week) or oral single dose MTX (25 mg once a week) regimen. Primary outcome was EULAR response (either good or moderate response) at 16 weeks [Table 1]. Analyses were intention-to-treat.
Table 1: Outcome between the two groups at 16 weeks (intention-to-treat using worst case imputation)

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Results: 50 patients (46 females) were randomized to split (n=26) or single (n=24) dose group. At 16 weeks, there was no significant difference in proportion of responders in split (84.6%) or single (66.4%) dose groups (p=0.14). Proportion of good responders was also not significantly different at 16 weeks (46.2%, 20.8%, p=0.06). However, there was significantly higher SDAI based remission (26.9%, 4.2%, p=0.048) and mean ΔDAS28-CRP (-2.3, -1.3, p=0.01) in the split-dose compared to single dose group. There was no significant difference in the ΔIHAQ and SDAI-LDA between groups. There was no difference in cytopenia, transaminitis or MTX intolerance between the two groups. No death or serious Adverse effects were seen. Serum MMP-3 declined in both groups but was not statistically different between the groups.

Conclusion: In this interim analysis there was no significant difference in primary efficacy outcome or tolerability or Adverse effects between split-dose compared to single dose oral MTX. However, some secondary outcome favored split-dose in terms of efficacy, which needs to be explored. Trial registration number: CTRI/2021/02/03136.


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Expert consensus on rheumatoid arthritis patients' profiles most likely to benefit from Baricitinib treatment

Chamaida Plasencia, Andrés Navarro1, Raimón Sanmartí2, José María Álvaro-Gracia3,4, Marta Comellas5, Clara Gabás-Rivera5, Sebastián Moyano6, Silvia Díaz6, Mercedes Núñez6, K B Rakesh7; Servicio de Reumatología, Hospital Universitario La Paz, Paseo de la Castellana, Madrid, 3Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Calle del Doctor Esquerdo, Madrid, 4Universidad Complutense de Madrid, Avenida Séneca, Madrid, 6Department of Medical, Lilly Spain, Av. de la Industria, Madrid, 1Servicio de Farmacia, Hospital General Universitario de Elche, Carrer Almazara, Elche, Alicante, 2Servicio de Reumatología, Hospital Clínic, Calle de Villarroel, Barcelona, 5Outcomes'10, Universitat Jaume I Parc Cientific Tecnológic i Empresarial Edificio Espaitec Avenida sos Baynat s/n, Castellón de la Plana, Castellón, Spain, 7Eli Lilly and Company (India) Pvt. Ltd., Gurugram, Haryana, India

Background: Baricitinib was approved in Spain for treatment of moderate-severe RA in adult patients with inadequate response/intolerance to disease-modifying antirheumatic drugs (DMARDs).

Objectives: Identifying profiles of RA patients that would obtain the greatest clinical benefit from baricitinib treatment.

Methods: Expert consensus based on Delphi methodology. The questionnaire used was developed based on review of scientific literature on baricitinib treatment in RA, a working group of experts and review of 3 rheumatologists, 1 pharmacist and 1 patient. It contained socio-demographic variables and 31 statements (58 items) regarding possible patient profiles for treatment. Participants expressed level of agreement with statements using 9-point Likert scale. Consensus was established when ≥75% participants scored a question in the range of disagree (items 1-3)/agree (7-9). Questions not reaching consensus were re-evaluated in a second round.

Results: 20 experts (mean age 52.8 [SD: 8.5] years, 55% women, 65% rheumatologists, and 35% hospital pharmacists) completed the 2-rounds. Consensus was reached in 69% of items. Participants agreed that baricitinib may be the treatment of choice over biologic DMARDs in patients (%agreement): refractory to ≥2 biologic DMARDs (100%), those in need of pain relief (85%), with intolerance to conventional DMARDs that would require monotherapy treatment (≥80%, except vs Anti-IL6), with advanced age (80%, except vs abatacept), with moderate-high levels of fatigue/ functional impairment (75%), with history of chronic or recurrent infections (≥75%, except vs abatacept), or with moderate-severe congestive heart failure (≥75%, except vs Anti-IL6/abatacept). Baricitinib may be the treatment of choice over other Janus kinase inhibitors in patients with: moderate liver failure (100%), advanced age (85%), or history of chronic/recurrent infections (75%).

Conclusions: There was consensus on the priority election of baricitinib over other drugs for RA treatment in a wide range of patient profiles, which may contribute to a better decision-making process and facilitate an individualized treatment selection.


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Expression of IL-33 and ST2 in peripheral blood mononuclear cells and serum of patients with active rheumatoid arthritis: A cross sectional study

Manju O Pai, S Venkatesh1, Praveen Kumar Singh2, Gaurav Badoni, Sarama Saha2, Pratima Gupta; Departments of Microbiology and 2Biochemistry, All India Institute of Medical Sciences, 1Department of General Medicine, Division of Rheumatology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Objective: There are a number of biochemical and inflammatory markers for rheumatoid arthritis (RA), a well-known autoimmune disease which attacks the joints, still there is a need to identify specific biomarkers that can be targeted for therapeutic purpose in these patients. This study attempted to study the levels of Interleukin 33 (IL-33) and its receptor ST2 in peripheral blood mononuclear cells (PBMC) and serum of active Rheumatoid Arthritis patients.

Methods: PBMC's and sera from 50 active RA patients and 50 healthy individuals were obtained. IL-33 and ST2 were measured using Real Time PCR with specific primers including internal controls using SyberGreen chemistry. Sandwich ELISA was done for protein expression studies for IL-33 and ST2.

Results: IL-33 and ST2 mRNA expression was significantly higher in patients with active RA, with estimated fold change of 3.533(ΔΔCt = -1.82121) and 4.172 (ΔΔCt = -2.15229) respectively, as compared to controls. Protein expression using sandwich ELISA revealed mean value of IL-33 to be significantly higher in RA patients than in controls (119.32±63.49 and 22.835±12.24 respectively). Similarly, an increase in serum protein of ST2 was also significantly higher in RA patients as compared to healthy controls (2434.328±834.60 and 705.22±310.05). Out of the total (nRA =50), 35 RA patients showed a hike in serum and mRNA expression of IL-33 which also showed correlation with other disease biomarkers like anti-CCP, CRP, DAS-28 CRP and HAQ-DI.

Conclusion: IL-33 and ST2 levels were elevated in both serum and PBMC's of RA patients and showed correlation with the disease activity. Therefore, IL-33/ST2L signalling represents to be involved in the pathogenesis of RA and also symbolizes to be a promising biomarker for RA that can be explored further for its therapeutic potential.


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Assessment of foot function and deformities in a cohort of patients with rheumatoid arthritis from Sri Lanka

Himantha Atukorale, H G H Udara, Dilusha Atukorale; Department of Rheumatology, District General Hospital, Matara, Sri Lanka

Background: Rheumatoid arthritis is known to cause deformities in the foot. Additionally, other foot defects could give rise to severe symptoms. These can impair foot functions and impact activities of daily living. Using footwear and appliances is considered beneficial to alleviate symptoms in patients with rheumatoid arthritis.

Objectives: The study aims to evaluate common foot deformities, functional impairment and symptoms in patients with established rheumatoid arthritis.

Methods: A sample of 72 patients treated for rheumatoid arthritis for over a year and who did not generally use footwear were recruited at Department of Rheumatology, Matara Hospital, Sri Lanka. Foot pain, assistive devices, surgeries, deformities, and Manchester Foot Pain and Disability Index (MFPDI) were studied using an interviewer-administered questionnaire [Table 1].
Figure 1: Reasons for avoiding footwear. Footwear was mostly avoided due to unawareness of its usefulness (54.2%), discomfort (18.1%), and slipperiness (16.7%)

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Table 1: The frequency and percentage of foot deformities in patients with rheumatoid arthritis

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Results: Majority (90.3%) were females. Mean (IQR) age was 60 (52-68)years. Mean duration of rheumatoid arthritis was 7.9 (SD- 6.44) years. Patients were on a mean number of 2 (SD-0.77) DMARDs. Mean BMI was 23.8kg/m2 (SD- 4.39). 25% and 37.5% was overweight and obese respectively. 52.8% had 1 or more comorbidities, the commonest being hypertension (20.4%). A majority of 68.1% reported foot pain, 41.7% had ankle pain, and 23.6% had forefoot pain. Only 2.8% have had foot surgeries. 12.5% were using assistive devices. Walking canes and modified shoes were used by 6.9%, and 5.6% respectively. 97.2% had one or more foot deformities.

Conclusions: Foot pain and deformities were frequently encountered among patients with rheumatoid arthritis necessitating proper evaluation of foot health. An awareness program is considered timely.

Duration of foot pain and number of DMARDs used positively correlated with MFPDI-pain intensity (p <0.05). Age of the patients positively correlated with MFPDI–personal appearance (p<0.05). The height of the patients negatively correlated with MFPDI-functional limitation (p<0.01). MFPDI-personal appearance positively correlated with functional limitation (p<0.05) and pain intensity (p<0.05).


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Evaluation of fracture risk by fracture risk assessment algorithm in patients of rheumatoid arthritis

Jainesh Jain, Rajnish Singh, Anil Taneja; ABVIMS and Dr. RML Hospital, New Delhi, India

Background: Rheumatoid Arthritis is a chronic autoimmune inflammatory disease with multisystemic, predominantly musculoskeletal involvement. RA patients are at high risk for osteoporosis and fractures. FRAX score is used to predict the 10 year risk of major osteoporotic and hip fracture.

Objectives: To assess bone mineral density (BMD) in patients with RA using DEXA scan (Dual Energy X-Ray absorptiometry) and calculate fracture risk using FRAX algorithm.

Methods: This cross sectional observational study conducted between 1 January 2021 and 31 May 2022 in tertiary care hospital in New Delhi enrolled 40 cases of RA. Demographic characteristics and anthropometric measurements were recorded, blood investigations including Rheumatoid factor (RF), Anti -citrullinated protein antibody (ACPA), erythrocyte sedimentation rate, C-reactive protein were done. DAS28-ESR was calculated. BMD was measured using DEXA scan and FRAX score was calculated using India specific FRAX calculator. Statistical analysis was done and p <0.05 was considered significant.

Results: Overall prevalence of osteoporosis was 55%. The mean of FRAX score for major osteoporotic fracture was 4.5 ± 5.18 % and for hip fracture was 2.11 ± 2.95%. Age, duration, swollen joint count, ESR, CRP, DAS28-ESR, and FRAX scores for major osteoporotic fractures and hip fractures were significantly higher in patients with osteoporosis. The independent predictors for higher FRAX scores are age, history of previous fracture, current smoker, history of glucocorticoid intake and T-score at femur neck [Table 1] and [Table 2].

Conclusions: Fracture risk using FRAX tool is higher in patients of Rheumatoid arthritis. There is high prevalence of osteoporosis in patients of RA. The independent predictors for higher FRAX scores are age, history of previous fracture, current smoker, history of glucocorticoid intake and T-score at femur neck.


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Prevalence and associations of extra-articular manifestations of rheumatoid arthritis: A cross-sectional study from a tertiary care center in South India

Harikrishnan Gangadharan, Akshay Akshay, Ronnie Thomas1, Josemon George; Departments of General Medicine and 1Community Medicine, Government Medical College, Kottayam, Kerala, India

Background: Extra-articular manifestations constitute a significant cause of morbidity and mortality in Rheumatoid arthritis (RA). There is a paucity of literature on the prevalence of extra-articular manifestations in South Indian patients with RA. We aimed to study the prevalence of extra-articular manifestations and their associations with various disease parameters in RA.

Methods: This was a hospital-based cross-sectional study conducted at a tertiary care teaching hospital in South India. The study population included the patients who attended the Rheumatology clinic/wards between October 2020 and August 2022. Out of the total of 271 patients who fulfilled the 2010 ACR/EULAR classification criteria of RA, 36 patients were excluded because of missing data. Out of the remaining 235 patients, the various extra-articular manifestations analyzed included anemia, SICCA, ILD, Rheumatoid vasculitis, serositis, neuropathy, subcutaneous nodules, bronchiectasis, and small airway disease. The comorbidities studied included diabetes mellitus, systemic hypertension, coronary artery disease, chronic obstructive pulmonary disease, hypothyroidism, and cerebrovascular accident. The various parameters like age, gender, disease duration, comorbidities, seropositivity, ESR, smoking, disease activity, and methotrexate use were compared between patients with extra-articular manifestations and those without extra-articular manifestations. Means were compared with unpaired t-test and medians were compared by Man Whitney U test. Categorical variables were compared by the chi-square test. A p-value < 0.05 was considered as significant.

Results: Among the 235 patients, 205 were female (87.2 %). The mean age of the study population was 51 ± 11.4 years and the median disease duration was 36 months (12-96). 221 patients (94 %) were seropositive. The median ESR was 52 mm/hour (29.6-80), and the median DAS28 ESR was 4.67 (3.80-5.64) [Table 1]. At least one extra-articular manifestation was seen in 98 patients (41.7%). The most common extra-articular feature was anemia followed by keratoconjunctivitis SICCA and ILD (14.5%, 10.6%, and 8.5% respectively). Univariate analysis demonstrated a significant association between median disease duration and the presence of extra-articular manifestations [60 ± 9.66 vs 36 ± 5.51 months, p= 0.013] [Table 2]. This association persisted in the multivariate analysis after adjusting for possible confounding factors. None of the other parameters were found to have a significant association with the presence of extra-articular manifestations.
Table 1: Univariate linear regression of various factors with fracture risk assessment score for major osteoporotic fracture

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Table 2: Univariate linear regression to find out factors affecting fracture risk assessment score for hip fracture

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Conclusion: To the best of our knowledge, this is the largest single-center cross-sectional study of extra-articular manifestations in Rheumatoid arthritis from India and we found extra-articular manifestations in a significant proportion of our RA patients irrespective of the age, comorbidities, seropositivity status, disease activity, or methotrexate use and had a positive association with disease duration.


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Ultrasound assessment of metacarpophalangeal synovial thickness with serological correlation in rheumatoid arthritis patients

Kunal Kishore, Pradeep, B A Muthanna, N K Jain, Bharat Hosur; Command Hospital (WC), Panchkula, Haryana, India

Background: Rheumatoid arthritis is a chronic inflammatory immune mediated disorder where synovial proliferation, pannus formation and bone erosions are histological hallmarks. High-resolution musculoskeletal ultrasound (MSUS) has been increasingly used in early diagnosis of the disease. While MSUS gray-scale (GS) usually identifies synovial proliferation, power Doppler (pD) may recognize active inflammation and neoangiogenesis. In addition, MSUS is also reliable for the detection of bone erosions as well as for the detection of subclinical synovitis and prediction of disease relapse and structural progression.
Table 1: Patient characteristics

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Table 2: Comparison of parameters between rheumatoid arthritis patients with extra -articular manifestations and without extra-articular manifestations

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Objectives: To perform high-frequency Ultrasound for measuring synovial thickness of (2nd, 3rd and 4th metacarpophalangeal joints (MCPJ)) in a group of patients with established rheumatoid arthritis (RA) (as per 2010 ACR/EULAR classification criteria for RA) and to determine its correlation with clinical and serological disease activity.

Methods: This Cross-Sectional Study was conducted in the Department of Radiodiagnosis, Command Hospital (WC), Panchkula from September 2020 to March 2022. It included 65 RA patients and 30 healthy volunteers who were age and sex matched. All subjects underwent high resolution US using a gray-scale US mode (8-18 MHz), (GE Healthcare LogicTM e), with a hockey stick probe (L8 – 18i-RS) with hands lying in prone position on the examination table. Both longitudinal and transverse scans were performed on dorsal regions of the 2nd, 3rd and the 4th MCP joints. The distribution of articular synovial blood flow was observed by color Doppler flow imaging (CDFI). The data obtained from the study was coded, recorded and analyzed using SPSS 21.0.

Results: The established Rheumatoid Arthritis patient who have raised CRP, positive RA factor and increased CCP values were found to have increased synovial thickness in MCP joints (2nd, 3rd and 4th). These patients have a characteristic distribution of synovial thickness with statistically significant maximal radial synovitis of the 2nd and 3rd MCPJ and ulnar synovitis of the 4th MCPJ compared to the normal individuals. However, the pD findings did not correlate with the increased serological marker and so with the increased synovial thickness. We also inferred that when transverse dorsal approach is utilised to scan MCPJ of the hands, the true synovial thickness can be measured without including synovial fluid.

Conclusion: Ultrasound measurement of synovial thickness is important tool to detect early rheumatoid arthritis patients from healthy individuals. The established Rheumatoid Arthritis patient who have raised CRP, positive RA factor and increased CCP values found to have increased synovial thickness in MCP joints (2nd, 3rd and 4th). These patients have a characteristic distribution of synovial thickness with statistically significant maximal radial synovitis of the 2nd and 3rd MCPJ and ulnar synovitis of the 4th MCPJ compared to the normal individuals. These finding are helpful in early diagnosis of seronegative RA or differentiate it from other common arthropathies.


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To use DAS 28 or CDAI in obese RA patients?

Sarthak Saxena, B K Kundu; Department of Medicine, ABVIMS and RMLH, New Delhi, India

Background: Measurement of disease activity by DAS28-ESR, DAS28-CRP, and CDAI has become an integral part of management of rheumatoid arthritis, by 'Treatment to Target' approach. With the exception of CDAI, the other three use inflammatory markers ESR and CRP to measure disease activity. Obesity is also known to increase inflammatory markers like CRP. We undertake this study to see if obesity confounds the disease activity measurement in RA leading to overestimation of disease activity.

Objectives: The primary objective was to examine the impact of obesity on commonly used disease activity indices and to differentiate whether the proposed impact is due to clinically assessed increased disease activity or solely due to increased inflammatory markers, thus overestimating the disease activity.

Methods: One hundred patients of RA (40 obese and 60 non obese) in remission or low disease activity as defined by CDAI, were divided into obese and non- obese groups based on Indian standards (BMI>25kg/m2). ESR and CRP were measured in both the groups. DAS 28-ESR and DAS28-CRP were calculated and compared using relevant statistical tests.

Results: DAS28-ESR and DAS-28-CRP scores were significantly higher in the obese subjects, despite both groups having comparable CDAI scores. Similar findings were also observed with inflammatory markers ESR and CRP, both being higher in obese patients.

Conclusion: We conclude that indices incorporating inflammatory markers, like DAS28 overestimate disease activity in obese RA patients. Treatment decisions regarding escalation or addition of DMARDs should be taken after considering the same. CDAI appears to be better suited for disease activity measurements in obese RA patients compared to DAS 28.


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Patient education survey in RA

S Pandya, M Bavaliya, P Belani, P Chotaliya, N Patel, A Parmar, V Sharma, R Shah, P Srivastava, D Raval, D Tanna; Rheumatology Association, Ahmedabad, Gujarat, India

Background: There is little data on how much what we communicate to our patients do they imbibe. We did a 10-question simple survey with our patients of RA.

Objectives: To see how much of information about RA that we share with our patients do they retain.

Methods: Informed consent was taken. We framed 10 simple questions for our patients – the questionnaire was in vernacular. There were 3 sets of questions: (1) Cause of disease – if they knew the name of their disease, what causes it and who should be the treating specialist. The 2nd set was on symptoms/organs involved if they knew other organs could be involved, whether it was ONLY joint disease or a systemic one and how long does the disease last and the last and 3rd set was on treatment aspect any relation to food they thought, if exercise was as important as medicines, if adverse effects of the drugs had been adequately explained and if their disease could be cured. We then analysed out of these 3 sets, from which one patient retained maximum information.

Results: 10 centers participated in this questionnaire-based study. The questionnaire was given to the patients of RA while they waited in the OPD. Out of the 3 sets of questions, more than 50% answered correctly the treatment aspect: most knew it could not be cured, that food had little relation, that exercises were as important and about adverse effects. The other two sets, a small proportion of patients knew correctly – especially the cause of the disease, about a third thought it was local joint disease and the disease would be long term/lifelong. More than 80% knew a rheumatologist should manage their disease and half knew the name of their disease.

Conclusion: More time needs to be spent with patients to explain nature of disease (RA), its cause, how long it would continue and likely complications and, in a language, that patients can understand and retain.


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Efficacy and safety of curcumin in maintaining remission during disease modifying anti rheumatic drug withdrawal in rheumatoid arthritis: Phase III at 52 weeks double-blind randomised placebo controlled trial

Padmanabha Shenoy1,2, Sreeja Bhatt1, Reshma Reji1, Anuroopa Vijayan1, Manju Mohanan1, Sakir Ahmed3, Pankti Mehta4, Aby Paul1; 1Centre for Arthritis and Rheumatism Excellence, 2Sree Sudheendra Medical Mission, Kochi, Kerala, 3Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, 4King George Medical University, Lucknow, Uttar Pradesh, India

Background: Curcumin, has been shown to have anti-inflammatory properties in observational studies. Despite the lack of well controlled studies, it is widely used as a dietary supplement by patients with Rheumatoid Arthritis (RA).

Objectives: The primary objective was to assess the efficacy of Curcumin to maintain remission at the end of 52 weeks in patients of RA when DMARDs were withdrawn. Secondary objectives were safety of curcumin, the estimation of serum curcuminoid levels and its correlation with flares.

Methods: In this single centre, patient and investigator blinded trial, adults with RA in sustained remission for more than 6 months were randomised in a 1:1 ratio to receive standardized Curcumin preparation twice daily or matching placebo. cs-DMARDS were tapered and stopped sequentially during each visit as per a fixed protocol.

Results: Of the 200 patients randomized 185 completed the study. Baseline characteristics were comparable between the groups. Per-protocol analysis included 92 participants in the curcumin group and 93 in the placebo group. At 52 weeks follow-up, flare-free survival was same between both groups [[Figure 1]; Log-rank test: p=0.76]. Adverse effects were similar across both groups with one each of infection and benign tumour in the curcumin group; and 2 new diagnoses of psoriasis in the placebo group. The curcuminoid levels, estimated in 72/92 in the Curcumin group and 70/93 in the placebo group showed a significantly higher serum level of curcuminoids in the interventional arm (Median [IQR]: 19.70 ng/ml [31-12] vs. 0.00 [0-3.0], P =<0.01), confirming the good bioavailability of the preparation used. Cox proportionate regression modelling showed that flares were independent of serum curcuminoid levels (Adjusted HR = 0.99 [95% CI: 0.969-1.0]; p=0.5) [Figure 2].
Figure 1: Flare-free survival in patients with rheumatoid arthritis on curcumin versus placebo at 52 weeks follow-up

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Figure 2: Adjusted Hazard ratios for flare in patients with rheumatoid arthritis as per cox proportionate regression modelling

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Conclusion: In RA patients who are in remission in whom csDMARDs were being systematically tapered, the addition of Curcumin, despite having a good bioavailability, did not affect the flare-free survival.


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Lack of awareness among patients and reluctance to refer to rheumatologists as major causes for rheumatoid arthritis diagnostic delay noted across India: Final Indian rheumatology association database report

S Chandrashekara, Padmanabha Shenoy1, Uma Kumar2, Sapan Pandya3, Alakendu Ghosh4, Apurva Khare5, Rajkiran Dudam6, Rudra Prasad Goswami2; ChanRe Rheumatology and Immunology Center and Research, Bengaluru, Karnataka, 1Centre for Arthritis and Rheumatism Excellence, Kochi, Kerala, 2Department of Rheumatology, All India Institute of Medical Science, New Delhi, 3Rheumatic Disease Clinic, Vedanta Institute of Medical Science, Ahmedabad, Gujarat, 4Department of Clinical Immunology and Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, 5Department of Medicine, LN Medical College and Research Center, Bhopal, Madhya Pradesh, 6HRC Hospital, Hyderabad, Telangana, India

Background: Early diagnosis and introduction of appropriate disease-modifying anti-rheumatic drugs (DMARDs) are the two key modifiable factors for achieving low-disease activity (LDA) or drug-free remission in rheumatoid arthritis (RA). For proactive intervention, it is paramount to understand the factors influencing the delay in achieving remission or LDA in a given population. The present study has attempted to address this objective using questionnaires developed by the Indian Rheumatology Association (IRA).

Methods: The data of patients with RA were extracted from the IRA database for 8 centers across India using a pre-designed questionnaire. The delay [Table 1] in the diagnosis was estimated as time of the first attributable symptom of arthritis or related symptom to defined clinical diagnosis. The reasons for delay in consulting a rheumatologist were analyzed as patient factors, referral factors and others. The patients who had a disease onset of <6 months upon consulting a rheumatologist, and had incomplete data and undetermined reasons were excluded.
Table 1: Probable causes for major diagnostic delay

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Results: The date of 4563 patients were extracted and the mean age of recruited subjects noted was 52.59±12.21. Among the selected participants [Table 2], increased female predominance was noted (n=3869). Majority of the subjects (80%) demonstrated ≥24 months as estimated time of delay in diagnosis from first symptom to diagnosis, followed by 6-24 months (12%), and <6 months (8%). When considering the patient factors, unawareness regarding the specialty was identified as the major cause for diagnostic delay (30.21%), followed by no belief in modern medicine (7%), belief of increased adverse events linked to modern care medicine (6%) and socioeconomic reasons (5%). Patients managed by different specialties (41%) were the major reason for referral-related diagnostic delay. Reluctant to refer to rheumatology (17%), not diagnosed as rheumatic disease (12%) and primary care physician not aware of the rheumatology speciality (12%) were the other referral-related reasons noted.
Table 2: Center wise data

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Conclusion: Only >10% of patients sought rheumatologist consultation within 6 months from the appearance of the first symptom of arthritis. The delay in diagnosis noted in the majority of the patients was attributed to the unawareness of the patients regarding the speciality. Patient management under different specialities added to the burden of delay and referral to rheumatology specialists. Pro-active intervention can reduce significant delay in referral.


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Huntingtin interacting protein 1 autoantibodies as a novel potential surrogate marker for rheumatoid arthritis: Pilot study

Surbhi, Ayushi Goel, Ved Chaturvedi, Sneha Verma, Sonia Rawat, Nirmal Kumar Ganguly, Shivani Arora Mittal; Sir Ganga Ram Hospital, New Delhi, India

Background: Rheumatoid Arthritis (RA), an autoimmune disease, primarily affects synovial joints but has systemic manifestations upon progression. Considering limited specific diagnostic and prognostic biomarkers, identifying the disease early and monitoring its progression is essential. Previous reports have shown that Huntingtin Interacting Protein 1 (HIP1) is over-expressed in rat synoviocytes, and its autoantibodies in sera of some cancers have diagnostic relevance. Here, we explored HIP1 and its autoantibody levels in sera of RA patients for their potential as surrogate markers.

Methods: Sandwich ELISA was performed with a human HIP1 ELISA kit (Bio String's, Pennsylvania, USA) and as per the manufacturer's instructions. A relative level of autoantibodies to HIP1 was detected using an in-house developed ELISA. Statistical analyses were performed using SPSS software version 26.0 (IBM Corp., Armonk, New York). A p< 0.05 at a confidence interval of 95% was considered significant.

Results: HIP1 expression was found similar in RA patients and controls. HIP1 autoantibodies were found significantly increased in RA patients (p=0.002) and were higher in patients with active disease, thereby correlating with disease progression (p=0.042).

Conclusion: We observed similar HIP1 levels in the sera of RA patients and controls. It is hoped based on HIP1 being a cytosolic protein. It is the first report indicating a humoral immune response against HIP1 in RA patients, correlating with active disease status. Further investigations in a larger cohort are required to validate this as a surrogate marker.
Table 1: Demographic features of healthy subjects and rheumatoid arthritis patients

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Table 2: Demographic features of healthy subjects and subgroups of rheumatoid arthritis patients

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Use of GSUS and PDUS in early rheumatoid arthritis

Manisha Ashwin Daware, Amritha, Pramodh, D G Santhoshkumar; Narayana Health City, Bengaluru, Karnataka, India

Background: The use of high frequency gray-scale (GSUS) and power Doppler ultrasound (PDUS) in rheumatology is cost effective modality and becoming an integral part in the management of patients in early diagnosis, assessing severity and activity monitoring in RA.

Objectives: To highlight the role of high-frequency gray-scale and power Doppler ultrasound in small joints of the hand in patients with early RA and correlate with clinical and radiographic findings.

Methods: During Study period, we examined 47 adult RA (18-65 years) patients with disease duration <2 years. GSUS and PDUS evaluation of commonly affected joints and other symptomatic joints like wrists, the second, third, and fifth MCP joints; second, third and fifth PIP joints is done. Semi-quantitative scoring was performed as per Szkudlarek's grading. Comparison is done between conventional radiography and ultrasound in detecting bone erosions.

Results: The mean age of patients included was 45.1 ± 10.6 years, including 68 % females and 32 % males. A total of 663 evaluated joints,which included 94 wrists, MCP2, MCP3, MCP5, PIP2, PIP3, PIP5 joints and 5 other symptomatic joints. Overall sonological findings in joints were, Erosion in 12.6%, Effusion in 12.8%,Synovial hypertrophy in 23.1%, PD/CD signal in 16.7%. Severity of each of these parameters were graded sonologically. GSUS was positively correlated with RF, bone erosion and duration of disease. No significant statistical correlation was found between the cumulative flow signal score and RF levels, disease duration.

Conclusions: Ultrasonography is more reliable than Conventional X-rays and a cost effective tool than MRI in diagnosing and assessing severity of joint involvement in RA in early stages.




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Correlation of depression, anxiety and quality of sleep with disease activity in rheumatoid arthritis: A study from tertiary care centre in India

Sahana Baliga, C Balakrishnan, Rohini Samant; P D Hinduja National Hospital and Research Centre, Mumbai, Maharashtra, India

Background: Rheumatoid arthritis (RA) is a chronic systemic inflammatory disorder involving joints. DMARDs are the best treatment offered to control disease activity in RA. However it is important to emphasize that sleep, depression and stress have also an important role to play in the disease management. Mental status of the patient along with quality of sleep should be carefully assessed along with disease activity. Through this study we attempt to find any correlation of depression, anxiety and insomnia with disease activity using DAS 28 score in RA.

Objectives: To find correlation of depression, anxiety and insomnia with disease activity in patients with RA.

Methods: It is a cross sectional study of 200 patients diagnosed with RA within 5 years of disease according to EULAR 2010 criteria attending OPD in our hospital. They assessed for depression, anxiety and insomnia using becks depression scale, becks anxiety scale and sleep quality scale respectively. Disease activity was assessed using DAS 28 score.

Results: Out of 200 patients, 29 (14.5%)patients had mild depression, 18 (9.0%)had moderate depression. 30 (15%) of them had mild anxiety, 25(12.5%) had moderate anxiety. 89 out of 200 patients in our study that is 44.5 % had disturbed sleep. The univariate analysis for depression showed significant association with number of drugs, pain scores, number of joints and high disease activity scores and multivariate for high disease activity. For anxiety univariate showed association with pain scores, number of joints, high disease activity and multivariate with increased number of joints. For sleep univariate showed association with fatigue, pain scores and high disease activity and multivariate with high disease activity [Graph 1] and [Graph 2].



Conclusion: Depression, anxiety, and sleep disturbance is significantly higher in patients with early RA. Assessment should also include assessment of the mental health. Early evaluation result in better outcomes and prevent unnecessary drug escalation.


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Clinical utility of soluble urokinase-type plasminogen activator receptor: A biomarker in rheumatoid arthritis patients in remission

Atul Kakar, Sangeeta Choudhury1, Pooja Rani1, Ved Chaturvedi2, Rizwana Hasan1; Departments of Medicine, 1Research and 2Rheumatology, Sir Ganga Ram Hospital, New Delhi, India

Background: Soluble urokinase plasminogen activator receptor (suPAR) is a new noval biomarker for the monitoring systemic inflammation. CRP and ESR are among the most frequently lab tests used to assess the disease progression and define remission in rheumatoid arthritis (RA). With this background, the present study aimed to assess the utility of serum suPAR levels for patients in clinical remission.

Methods: Enrolled 25 RA patients with clinical remission (ACR criteria) and measured serum suPAR levels using suPARnosticR ELISA Assay kit (ViroGates, Denmark) and correlated with CRP, ESR and DAS28.

Results: The mean age was 51.67 ± 12.93 (range 29- 69 years). Tender joint and swollen joints were seen in 16.7% and 8.3 % respectively. The mean ± SD of CRP was 2.86 ± 0.3, DAS28 score was 2.15 ± 0.59 and ESR was 7.02 ± 0.25. Nonparameteric analysis (Spearman's rho test) showed significant correlation (p=0.008) with DAS28 and ESR. As per Mann-Whitney test, the level of serum suPAR amongst RA remission patients were significantly higher (p<0.01) compared to healthy individual (mean ± SD, 7.27 ng/ml vs. 3.59 ng/ml). The ROC curve observed the AUC as 0.954 wherein sensitivity was 83.3% and specificity was 100%, NPV of 84% and PPV of 100%. Fisher's Exact test identified a significant number of patients who had suPAR >4.46 ng/ml. Among the co-morbidities, only hypertension was observed to be significantly associated with suPAR levels (2-tailed Wilcoxon test; p=0.04).

Conclusion: High suPAR levels among patients with clinical remission of RA demonstrates baseline inflammatory status despite normal to mild range of ESR, CRP and/or DAS 28 score. SuPAR is a prognostic biomarker that can aid in better therapeutic management of RA in remission patients.


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Drug safety and pregnancy outcome in Indian patients with rheumatoid arthritis

Parshant Aggarwal, Bharti Aggarwal, Vikas Gupta1, Palak Gupta1; Punjab Rheumatology Immunology Centre, 1Department of Clinical Immunology and Rheumatology, Dayanand Medical College Hospital, Ludhiana, Punjab, India

Background: Due to ethical issues conducting controlled trials to assess safety, teratogenicity of medicines to treat Rheumatoid Arthritis (RA) is not feasible and pregnant women are excluded from drug trials. Hence data concerning safety of arthritis medicines in RA is limited. To our knowledge no study is available from India regarding pregnancy outcome and drug safety in RA patients.

Objectives: Analyze safety of arthritis medicines and pregnancy outcome in Indian women suffering from RA.

Methods: Retrospective analysis of RA women with pregnancy treated at Punjab Rheumatology Immunology Centre, Ludhiana and DMC, Ludhiana between 2012 to 2021. Patients were classified as RA based on ACR/EULAR 2010 criteria. Compliance to treatment and additional medication usage was self-reported by the patients. Delivery of healthy baby was counted as successful while spontaneous abortion, MTP due to fetal abnormality were counted as adverse pregnancy outcome.

Results: 74 patients (Mean age 31.3 years) and 79 pregnancies were evaluated. 65 were successful. 10 patients had 11 spontaneous abortions out of whom 4 subsequently had successful pregnancy. Anticardiolipin antibodies were negative in 7, not available in 3. Patient with 2 spontaneous abortions had a successful pregnancy following low molecular weight heparin treatment throughout pregnancy despite negative anticardiolipin antibodies. 3 patients underwent MTP due to antenatal fetal defects [Table 1]. Most commonly used medicines during pregnancy were Sulfasalazine(SSZ), Hydroxychloroquine (HCQ), Low dose Prednisolone, Azathioprine(AZA) [Table 1]. No specific teratogenicity pattern was identified. 3 patients without Methotrexate (MTX) and 1 patient without MTX+Leflunomide had successful pregnancy.
Table 1: Drug list and adverse outcomes

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Conclusions: To our knowledge this is the first study evaluating drug safety and pregnancy outcome in Indian RA patients. Most RA patients can have successful pregnancy. SSZ, HCQ, Azathioprine and low dose prednisolone are safe during pregnancy. Limited data indicates patients can have successful pregnancy without observing MTX washout but more data is required.


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Development, validation and performance of a patient knowledge questionnaire for rheumatoid arthritis in Indian patients

Ranjan Gupta, Rudra Prosad Goswami, Manshi Yadav, Sandhya Saini, Anju Mohan, Vanamail Perumal1; Departments of Rheumatology and 1Obsterics and Gynecology, All India Institute of Medical Sciences, New Delhi, India

Background: Long-term outcomes in rheumatic diseases can be improved by enriching patients' knowledge, beliefs and perception about their disease.

Objectives: To assess the impact of an information course in improving the above elements among patients with rheumatoid arthritis (RA) using a self-prepared questionnaire.

Methods: A self-prepared questionnaire (Hindi/English) containing 24 multiple choice or true-false type questions with single correct answer assessing patients' knowledge about RA in 3 domains (a. etiology, disease process, signs and symptoms b. drug therapy and monitoring c. joint protection, exercise, coping) was prepared. All questions had an item-level content validity index (I-CVI) of at least 0.9. The scale-level content validity index based on the universal agreement method (S-CVI/UA) was 0.8 for the whole questionnaire as assessed by 6 experts. All questions had an option of 'I don't know'. Frequency data were compared using McNemar test for paired nominal data and kappa statistics for ordinal data. Student's paired t-test was used for comparison of mean scores to see the impact.

Results: The baseline scores (mean±SD: 6.56±4.33) of patients (n=126; F:M=117:9) was poor [Table 1]. The option 'I don't know' was exercised by a median 42.2% times before the information course which reduced to 12.5% after the course. After the information course, all the questions recorded a higher absolute number of correct responses as compared to before the course [Figure 1] and this increase in correct responses in 21 out of 24 questions achieved statistical significance [Table 1]. The mean (±SD) score for the questionnaire improved from 6.56 (±4.33) to 16.19 (±5.15) (p<0.001) which was statistically significant irrespective of the socio-economic classes and education status of the patients.
Figure 1: Proportions of question-wise pre-course incorrect responders (blue) who responded correctly in post-course assessment (red)

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Table 1: Question -wise assessment of responses before and after the information course and the statistical significance of the change

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Conclusion: Indian patients with RA have a poor knowledge base about their disease which can be improved significantly by short information courses. The information course is equally effective across all the socio-economic classes irrespective of their educational status.


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Clinical and economic burden of Herpes Zoster in patients with rheumatoid arthritis: A retrospective cohort study using administrative claims

Ashfaque Shaikh, David Singer1, Philippe Thompson-Leduc2, Sara Poston1, Deepshekhar Gupta3, Wendy Y Cheng4, Siyu Ma1, Francesca Devine5, Alexandra Enrique3, Mei Sheng Duh4, Jeffrey R Curtis6; GSK, India, 1GSK, Philadelphia, PA, 3Analysis Group Inc., Menlo Park, CA, 4Analysis Group, Inc., Boston, MA, 5Analysis Group, Inc., New York, NY, 6University of Alabama at Birmingham, Birmingham, AL, USA, 2Analysis Group, Inc., Montreal, QC, Canada

Background: Herpes zoster (HZ) incidence is higher in patients with rheumatoid arthritis (RA) than in the general adult population.

Objectives: This study sought to estimate the clinical and economic burden posed by HZ in patients with RA.

Methods: A retrospective cohort study was conducted using data from an administrative claims database (Oct2015-Feb2020). Patients with HZ and RA (RA+HZ) were identified using ICD-10-CM diagnosis codes. RA was defined as ≥2 RA diagnoses ≥6 weeks apart and ≥3 months of DMARD treatment. A comparator cohort with RA but without HZ was identified. Index was either the HZ diagnosis (RA+HZ) or a randomly selected date (RA-only). Patients had at least 12-month medical and pharmacy benefit enrolment before (baseline) and after (follow-up) index. Outcomes included healthcare resource use (HCRU) and total, medical, and pharmacy costs during the 12-month follow-up. Generalized linear models were used to estimate differences in outcomes between cohorts.

Results: Mean age (± standard deviation [SD]) of the 1,866 RA+HZ and 38,846 RA only patients was 68 (±12) years and 66 (±13) years, respectively. Most patients were females. Higher proportions of RA+HZ patients used JAK inhibitors or systemic steroids at index compared to RA only patients [Table 1]. Baseline mean ± SD total costs were $ 52,625 ± 67,774 and $ 46,332 ± 65,480 in the cohorts with and without HZ, respectively. Hospitalizations and emergency department (ED) visits occurred more often in the RA+HZ cohort with an adjusted incidence rate ratio of 1.16 (95% CI: 1.04-1.30) for hospitalizations and 1.34 (1.21-1.47) for ED visits. Medical costs were higher in the RA+HZ cohort, with an adjusted cost difference of $3,428 (446-6,781) [Table 2].
Table 1: Baseline demographic, clinical, and cost information for patients with rheumatoid arthritis and Herpes zoster and patients with rheumatoid arthritis only

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Table 2: Healthcare resource use and costs during 12 months after index date for patients with rheumatoid arthritis and Herpes zoster and patients with rheumatoid arthritis only

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Conclusions: Patients with RA and HZ had higher HCRU and medical costs than patients with RA only in the year following an HZ diagnosis after adjusting for baseline difference between cohorts.

Funding: GlaxoSmithKline Biologicals SA.


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Assessment of the levels of 14-3-3 eta protein, a novel biomarker, in patients with rheumatoid arthritis and its association with disease activity

Raghuraj Chawla, Piyush Jain, Anuradha1; Departments of Medicine and 1Microbiology, ABVIMS and Dr. RML Hospital, New Delhi, India

Background: Rheumatoid arthritis (RA) is a chronic multisystemic disease diagnosed by clinical features and inflammatory and immunological markers. But none of the currently available biomarkers is 100 % sensitive or specific for RA and there is a need for some new biomarker that can aid in diagnosing RA and in monitoring therapeutic response.

Objectives: To assess the 14-3-3η (eta) protein levels in RA patients, to compare its positivity with that of Rheumatoid factor (RF) and Anti-citrullinated protein antibody (ACPA), and to see the association between 14-3-3η protein levels and disease activity.

Methods: This comparative observational study conducted between 1st January 2021 and 31st May 2022 in a tertiary care hospital in New Delhi enrolled 78 RA cases and 78 age and sex-matched healthy controls. Demographic characteristics were recorded and blood investigations including Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), RF, ACPA, and 14-3-3η protein levels were done. DAS28-ESR scoring was done. Statistical analysis was done and p < 0.05 was considered statistically significant.

Results: RF, ACPA, ESR and CRP positivity was significantly higher in RA patients as compared to controls. The mean 14-3-3η protein level was 7.61 ± 2.55 ng/ml in cases {7.64 ± 2.62 ng/ml in seropositive cases and 7.18 ± 1.62 ng/ml in seronegative cases (p =0.673)} and 0.73 ± 0.43 ng/ml in controls (p <0.001) [Table 1]. Levels were higher in patients with higher disease activity (DAS28-ESR) (p=0.020) [Table 2]. The sensitivities of 14-3-3η protein, RF and ACPA were 100%, 88.5%, and 73.1% respectively. 14-3-3η protein increased the sensitivity of RF or ACPA when combined with them.
Table 1: Comparison of 14-3-3η protein levels between cases and controls

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Table 2: Association of 14 -3-3η protein levels with disease activity (disease activity scores 28 -erythrocyte sedimentation rate score)

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Conclusions: 14-3-3η protein levels are significantly raised in both seropositive and seronegative RA patients than in healthy controls. It correlates with disease severity (as measured by DAS28-ESR). It increases the sensitivity of RF and ACPA in diagnosing RA and may be included as an additional test while evaluating RA patients.


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A retrospective analysis of patients of palindromic rheumatism presenting to our OPD and their outcome

S Pandya, R Solanki, T Parikh; Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: There is scarce data on palindromic rheumatism (PR) from the subcontinent. [1,2] We wanted to look at baseline features and outcome of our group of patients over years of follow up.

Objective: Descriptive analysis of patients presenting to our OPD and outcome over years of follow up.

Methods: Retrospective analysis of data collected from April 2012 to August 2022 was done. Descriptive statistics were applied and we looked at the last follow up how many had decrease in frequency of attacks. Informed consent was taken.

Results: [Table 1] shows the values.

Conclusions: Patients with PR presented on an average at 3 years. They had severe attacks at presentation and of data available on follow up (50 patients), most had > 80% improvement at last follow up (over approximately 2 years) being treated with MTX and HCQ as the most common modality.


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Concordance between rheumatoid factor and anti-citrullinated protein antibodies in patients with rheumatoid arthritis and their association with risk factors

Shweta Agarwal, Ausaf Ahmad, Nida Khan; Integral Institute of Medical Sciences and Research, Integral University, Lucknow, Uttar Pradesh, India

Background: Presence of Rheumatoid factor (RF) and anti-citrullinated protein antibodies (ACPA) is important for diagnosis of Rheumatoid arthritis (RA). We wanted to see if we can stick to only one test in our resource constraint population and if presence of these antibodies is associated with various risk factors implicated in development of RA.

Objectives: To study the concordance between RF and ACPA in patients with RA and their association with age, gender, smoking and BMI.

Methods: Demographic profile of 210 patients, diagnosed with RA, based on 2010 ACR/ EULAR classification criteria of RA, was studied in the form of age, gender, tobacco use and body mass index (BMI). RF and ACPA were measured in all the patients. Both the antibodies were studied for concordance between themselves and their correlation with age, gender, tobacco use and BMI.

Results: Among the 210 patients, 68.6% were below 50 years of age and 85.7% were female. Only 3.3% were daily smokers while 10% were tobacco chewer and 76.7% did not use tobacco in any form. 74.3% patients were found to be obese [Table 1]. [Table 2] show that 116 (55.3%) patients displayed positive results for both RF and ACPA. There were 17 (8.1%) patients who had negative results for both. However, there were 77 (36.6%) individuals (i.e. 36 + 41) for whom the both tests could not agree on their results. Both the antibodies (RF & ACPA) were not found to be significantly associated with age, gender, smoking or BMI.
Table 1: Palindromic rheumatism demography

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Table 1: Demographic profile of patients with rheumatoid arthritis

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Table 2: Comparison between rheumatoid factor and anti-citrullinated protein antibodies

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Conclusion: It is important to have both RF and ACPA done in patients with RA as in one-third of the patients there is no concordance between the two tests. Age, gender, smoking and BMI of the patient did not influence the presence of antibodies in our patients.


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Cost-effective analysis of generic targeted synthetic disease-modifying anti-rheumatic drug: Tofacitinib in rheumatoid arthritis

Ashit Syngle1,2, Inderjeet Verma3, Kanchan Chauhan1, Sapna Patiyal1, Devaansh Syngle4; 1Healing Touch City Clinic, 4Healing Touch Foundation, Chandigarh, 2Fortis Multi Specialty Hospital, Mohali, Punjab, 3M. M. College of Pharmacy, Maharishi Markandeshwar (Deemed to be University), Mullana-Ambala, Haryana, India

Background: Safety and efficacy of tsDMARD tofacitinib has been well demonstrated in Indian RA patients with rheumatoid arthritis. Tofacitinib has 0.08% of market value share [as against 0.06% for methotrexate] of anti-rheumatic drugs in Indian Pharmaceutical market. However, the cost-effectiveness of tofacitinib in Indian RA patients and generic tofacitinib has not been evaluated.

Objectives: Perform pharmacoeconomic analysis of generic tofacitinib in rheumatoid arthritis patients in India.

Methods: A prospective, observational study was conducted in 55 consecutive RA patients meeting 2010 Rheumatoid Arthritis Classification Criteria with active disease and an inadequate response or intolerance to conventional disease-modifying antirheumatic drugs randomized to receive tofacitinib 5 mg bd as an add on therapy. All study patients attended the rheumatology OPD with 3 months follow-up. Average cost effectiveness analysis was done by taking HAQ-DI score as a measure of effectiveness. Total cost estimation included cost of the treatment, monitoring cost and adverse effect management.

Results: RA patients included in study had established disease with 18.18% males and 81.81% females and 49% were seropositive. Majority of the study RA patients (89%) were on a background regimen of two synthetic DMARDs (MTX and HCQ-80%, MTX and LEF-5.45%, SSZ and HCQ-3.63%,) and 11% of the RA patients were combination of three sDMARDs. After 3 months treatment with generic tofacitinib and other sDMARDS, the DAS-28 score improved from 5.31 ± 2.16 to 3.81 ±1.21, p≤0.001 and the mean disability index estimated by HAQ-DI reduced from 1.39 ± 0.60 to 0.62 ± 0.37, p<0.001. The direct medical cost of treatment of RA per month is 3032.81 rupees [Figure 1]. The average cost effectiveness ratio for the treatment with generic tofacitinib and continuing the use of csDMARDs were Rs 3,938.71 to gain or improve one unit of HAQ-DI score.
Figure 1: Distribution of total direct medical cost

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Conclusions: Treatment with generic tofacitinib is effective to control the disease activity and improves disability in RA. Incorporating generic tofacitinib into the csDMARD treatment sequence in active RA patients who have inadequate response or intolerance to csDMARDs is effective to improve the disability index. These results, reflecting the additional costs associated with deteriorating health condition, support the importance of using generic tofacitinib.


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Factors predicting early achievement of remission in treatment-naïve rheumatoid arthritis patients receiving conventional DMARD

Shruthi Desai, S Chandrashekara, Sayid Fahad Nazir; ChanRe Institute of Rheumatology and Immunology, Bengaluru, Karnataka, India

Background: In early RA, remission is particularly important and early intensive treatment can halt or substantially reduce subsequent disease progression. The present study evaluated the factors predicting failure to achieve remission in DMARD naïve RA patients receiving double and triple DMARDs at 3 months and 12 months. Both machine learning algorithm and conventional logistic regression were used to develop a model and to analyze their consistent predictive potential.

Objectives: To identify the clinical variables that could predict achievement of remission in treatment-naïve rheumatoid arthritis patients at 3 months and 1 year.

Methods: The retrospective study recruited patients who fulfilled ACR-EULAR 2010 RA criteria and were managed by standard care with regular monitoring of disease activity and conventional DMARD. DAS-28 CRP of 2.6 was considered as target to be achieved. The factors predicting the achievement of target at two points were analysed (end of 3 months and 12 months) using logistic regression and machine learning methods (decision-tree model).

Results: Among the total of 219 RA patients involved, 55.7% achieved overall remission. Male gender, shorter disease duration, and CRP<30 at presentation were associated with remission [Table 1]. Presence of comorbidities- T2DM and thyroid disease, high inflammatory markers, and higher tender and swollen joint counts negatively predicted the remission. Logistic regression and decision-tree models accurately categorized 95% of the patients Please see attachment given there are 39 articles along with sequence to follow [Figure 1].
Figure 1: Decision tree model by machine learning model - at 1 year

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Table 1: Predictors of achieving remission at the end of 1 year of csDMARDs

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Conclusion: The female sex, longer duration of illness, presence of comorbidities, high inflammatory markers, high tender joint count, and high baseline DAS score were the key factors adversely impacting the achievement of LDA at 1 year. Targeting this group of patients with intense, individualized therapy and rapid escalation of treatment would facilitate higher remission rates and improve overall disease progression. Machine learning models could be incorporated to predict the response of remission outcomes and categorize them accurately to aid in treatment.


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A clinical study on rheumatoid arthritis with reference to ultrasonographic evidence for early diagnosis

R R Marak, Kabita Brahma; Department of Medicine, Assam Medical College, Dibrugarh, Assam, India

Background: In patients with rheumatoid arthritis, regular monitoring of joint inflammation and disease activity is important in evaluating treatment response and disease outcome. Synovitis has important role in RA. So monitoring of response to therapy should mainly focus on synovitis.

Objectives: To assess ultrasonographic evidence of synovitis in patients of rheumatoid arthritis as an aid for treatment of disease.

Methods: 70 patients with RA were included in the study. 28 joints which includes bilateral glenohumeral, elbow, wrist, metacarpophalangeal (MCP), proximal interphalangeal (PIP) of hands, and knee joints were assessed for swelling and tenderness. For every patient DAS 28 ESR and CDAI were calculated. An Ultrasound examination was done in 28 clinically examined joints. Each of 28 joints were examined for presence of synovial hypertrophy (SH), power Doppler (PD) signals and effusion. From this, composite US measures of synovial disease were made: SH joint count (SHJC), Effusion Joint Count (EJC), Power Doppler Joint Count (PDJC).

Results: Joints with tenderness showed significantly less PD scores than other groups. SH and PD signals were detected in 33.2% and 26.5% of nil group respectively SHJC showed moderate correlation with TJC and high correlation with SJC, ESR, CDAI, DAS28-ESR, EGA and PGA. PDJC showed moderated correlation with TJC and high correlation with SJC, EGA, DAS-28, ESR.

Conclusion: Ultrasound count for SH and PD relate significantly to DAS 28-ESR and CDAI and their components.


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Treatment adherence of patients with rheumatoid arthritis during COVID-19 pandemic

Syamasis Bandyopadhyay, Susobhan Mondal; Apollo Gleneagles Hospital, Kolkata, West Bengal, India

Background: COVID-19 impacted not only people's lives but also slowed down the healthcare delivery system and supply chain leading to a global drug shortage. According to the Ministry of Statistics, India' s growth in the year 2020 went down by 3.1% because of the pandemic, which impacted patient's capacity to continue with the expenditure related to chronic disease management. Rheumatoid arthritis (RA) for a patient comes with a out-of-pocket high cost long term immunosuppressive medicine and increased chances of secondary infections leads to non-adherence of patients.

Objective: The current study is to observe the adherence to Janus Kinase (JAK) inhibitors in a hospital-based rheumatology service in Eastern India during the COVID-19 pandemic period.

Methods: Data of the patients enrolled physically and electronically under active follow-up in the Rheumatology Outpatient Department (OPD) of the hospital were analyzed. The patients with a confirmed diagnosis of RA, receiving JAK inhibitors for 6 months or more were included in the study from 21st March 2020 to 31st July 2020. A questionnaire was also administered to these patients to understand the impact of COVID-19 on the treatment of RA. Data related to demographic features, clinical, laboratory, drug history, and current treatment were collected and statistically analyzed.

Results: Out of the total 42 patients (aged 38-76 years) who received JAK inhibitors, 24 (6 were COVID positive) were seen with the OPD during the COVID-19 pandemic. In our study, a higher proportion of patients with an annual income of INR 1M-1.5M had a 15% income decrement [Figure 1], though the patient adherence to JAK inhibitors was high compared to biologics, even in the patients who faced up to 25% reduction in annual income. Out of 24, only 4 patients stopped the treatment with JAK inhibitors due to the limited availability during the initial period of the lockdown. Overall patient adherence to JAK inhibitor treatment was 85% and was higher compared to the biologics (previous data). There was higher non-adherence in the biologic group at lower-income slabs (5-10 Lacs and 10-25 Lacs group) than in the higher income slabs, compared to JAK inhibitors inspite of better availability. Higher-income groups showed lower non-adherence in both groups.
Figure 1

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Conclusion: In the milieu of the COVID-19 pandemic, the treatment adherence in patients with RA was driven by the cost and availability of the medication amidst the pandemic. The association of injectable biologics with higher immunosuppression in patients perception during pandemic also affected the treatment adherence in patients. Thus it can be concluded that patient perception and availability were the main driving factor in adherence to RA therapy.


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Iguratimod in rheumatoid arthritis the new molecule with good safety profile: A real life experience of 1853 patients

V N Nagaprabu, P Velammal1, A Gayathri, R Saranya, K Aswathi; Sakthi Rheumatology Centre Pvt Ltd, 1PSG Institute of Medical Sciences and Research Centre, Coimbatore, Tamil Nadu, India

Background: Iguratimod is one of the newer DMARDS with limited evidence in western literature of its efficacy. In view of its analgesic effects along with evidence from other studies it seems to be a promising drug therapy. The definitive role of Iguratimod in treating patients with rheumatoid arthritis needs to be explored.

Methods: All RA patients who were treated with Iguratimod during the time period September 2020 to December 2021 were included for analysis. Patients demographics, available lab parameters, details of drugs received change in steroid doses were noted.

Results: A total of 1853 patients received Iguratimod during this time period. Mean age of the group was 50.71 (11.80) years and female: male ration was 1609:244. and mean disease duration was 3.75 (5.89) years. Of the total 1853 patients there were total of 1497 patients who continued to have Iguratimod. in this group patients with less than 3 months of follow-up were excluded so there were a total of 1093 patients for final analysis. The mean age in this group was 51.19 (11.64) years and F:M ratio was 955:138 with mean disease duration 3.68 (5.89) years. There were no significant change in the liver function test, ESR, blood sugar values but there was significant reduction in the rheumatoid factor levels and the dose of steroids in patients who were continued with Iguratimod [Table 1]. 333 patients have stopped Iguratimod for various reasons commonest being persistent pain in 189 followed by Gastric intolerance (44), 35 (not affordable), 27 (no reason), 25 better, 7 itching, palpitations in 5, infection in 1.
Table 1: Baseline characteristics of patients included

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Conclusion: Iguratimod leads to significant reduction in rheumatoid factor levels and is well tolerated with no major adverse effects.


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Rituximab versus leflunomide in combination with methotrexate in patients with active rheumatoid arthritis despite methotrexate treatment: An open label randomised controlled trial

Rashmi Roongta, Geetabali Sircar, Dipendranath Ghosh, Hiramanik Sit, Parasar Ghosh; Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Background: Active rheumatoid arthritis (RA) despite methotrexate (MTX) monotherapy is seen in 40-50% patients. The current ACR guidelines recommend adding a biologic or targeted synthetic DMARD to MTX. EULAR guidelines recommend a combination of DMARDs (most commonly- MTX, sulphasalazine and hydroxychloroquine). In a resource limited country like ours, adding a bDMARD to every MTX failure is implausible. Often, leflunomide is added in such cases.

Objectives: We aimed to study if leflunomide with MTX combination is comparable to rituximab (RTX) and MTX combination in patients with MTX failure.

Methods: This open label, randomised controlled trial, was carried out in patients having moderate to high disease activity despite MTX therapy (CTRI - REF/2021/04/042755). Patients were randomised to receive either leflunomide (10-20 mg/day) or RTX (2 doses of 1 gm each, 2 weeks apart) along with background MTX. The primary outcome ACR20 response at 24 weeks and secondary outcomes were ACR50 and 70 responses at 24 weeks.

Results: There were 21 patients in each arm. Baseline characteristics were similar in both groups [Table 1]. The ACR20 response at 24 weeks was achieved by 14 (66.6%) and 16 (76.19%) patients respectively in the leflunomide and RTX arms (p=0.734). ACR50 response was achieved by 11 (52.38%) patients in both groups and ACR 70 response by 4 (19%) and 7 (33.3%) in the leflunomide and RTX arms [p= NS in both, [Table 2]]. Two patients in the RTX arm had infusion reactions which were managed with anti-histamines and steroids. No infections were reported in any group. Mild transaminitis was noted in four patients receiving LEF but none required drug withdrawal.
Table 1: Baseline characteristics of both arms (n=21)

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Table 2: Responses in both groups at Week 24 (n=21)

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Conclusion: LEF was as efficacious as RTX for the achievement of ACR20/50/70 responses at 24 weeks. LEF can be considered as an add-on option to methotrexate instead of more expensive biologic agents in the case of methotrexate refractory RA. Larger studies are needed to confirm this hypothesis.


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Comparative efficacy and safety of interleukin-6/JAK/STAT pathway inhibitors in rheumatoid arthritis: Systematic review and network meta-analysis

Arindam Banerjee, Moumita Chatterjee1; Department of Mathematics, Indian Institute of Technology, Kharagpur, 1Department of Mathematics and Statistics, Aliah University, Kolkata, West Bengal, India

Background: Treatment for rheumatoid arthritis (RA) has diversified in recent years with introduction of multiple small molecule inhibitor of the interleukin-6 (IL-6)/JAK/STAT pathway. However, recent studies have raised questions on cardiac safety. No systematic reviews are available on the comparative efficacy and safety of these drugs in RA.

Objectives: To indirectly compare the efficacy and safety of IL-6/JAK/STAT pathway inhibitors (tocilizumb, tofacitinib, baricitinib, filgotinib and upadacitinib) among patients with RA.

Methods: PubMed Database was searched to identify randomised controlled trials on RA and any of the above drugs confined to a predefined inclusion and exclusion criteria. A systematic review and network-meta-analysis was performed on the included studies on efficacy (ACR20 response) and safety signals (infections, MACE and malignancy).

Results: Overall, 43 trials were included (five in naïve patients (n=2342), 23 in disease modifying drug inadequate-responders (DMARD-IR, n=8734) and seven in biologics inadequate-responders (BIR, n=1878). Among naïve patients, tocilizumab, tofacitinib, baricitinib and upadacitinib monotherapy data were available, and all were superior to methotrexate. Data on all the drugs were available for DMARD-IR patients in combination with methotrexate, all of which were superior to methotrexate but regarding monotherapy tocilizumab (Odd's Rratio (OR): 2.52, 95% confidence interval (CI): 0.83-7.62) were similar to methotrexate. Data on BIR were available for tocilizumab, tofacitinib, baricitinib and upadacitinib, all of which were superior to control. Serious infections were higher in tocilizumab group (OR: 1.73, 95% CI: 1.01-2.94); herpes zoster infections were higher with baricitinib (OR: 3.58, 95% CI: 1.2-10.7) and tofacitinib (OR: 3.05, 95% CI: 2.28-4.09). MACES were similar among all. Rates of malignancy were higher with tofacitinib (OR: 2.94, 95% CI: 1.56-5.53).

Conclusion: In DMARD-IR population, combination with methotrexate showed higher effect sizes. No increased risk of MACE was observed for any of the drugs. An increased risk of malignancy was seen with tofacitinib.




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Study of disease activity and disability index in rheumatoid arthritis in a tertiary care rheumatology clinic

Deepika Ravi, Aarti Zope, Ajit Ghadge, Amruta Bagul, Amruta Digole, Lalana Jadhav, Yojana Gokhale; Department of Medicine, Rheumatology Services, L.T.M.M.C, Mumbai, Maharasthra, India

Background: The aim of RA treatment is changing from early aggressive treatment to treat-to-target, where target is remission/low disease activity (LDA). In various trials, on an average 30% (20 to 40%) patients achieved remission when defined by DAS28-ESR scores. But in actual practice, situation is unlike clinical trials, for e.g. in QUEST-RA Study, multi-centre trial performed across various countries, overall patients had average DAS28-ESR score 4.0.

Objectives: To find out proportion of patients who achieve target i.e. remission/LDA. To find out average disease activity and disability index (DI) of RA patients who were on DMARDs for at least 6 months. To study social factors affecting disease activity/poor disease control.

Methods: Patients diagnosed as RA by ACR/EULAR 2010 classification criteria, on DMARDs for atleast 6 months were included. It was cross-sectional study done over 2 months in tertiary care Rheumatology clinic. Disease activity using DAS28-ESR and DI using Indian-HAQ was calculated at one visit. Additional data regarding compliance, use of alternative medicine, level of education, per capita income and distance from hospital was also noted. Statistical methods- Categorical variables like compliance, use of alternative medicine, education <10 th std, per capita income </= INR 4000, distance from hospital >40 km were analysed using Chi square test. Sample size- 78.

Results: Out of 78 patients, females were 71 (91%), males were 7 (9%), F:M=9:1; average age was 40 years (23-68); average disease duration was 5 years (1-26). Average DAS28-ESR score was 3.74 (0.97-6.72). Remission and LDA was seen in 38.46% and high disease activity (HDA) and moderate disease activity (MDA) was seen in 61.54%. Compliance to treatment was seen in 66.7% of patients, among them remission/LDA was seen in 28 (53.8%) and MDA/HDA in 24 (46.2%). This study revealed statistically significant correlation of compliance and disease activity by DAS28-ESR (p=0.004). Use of alternative medicine was seen in 12 (15.4%) patients. Among them, 8.33% had remission/LDA and 91.67% had MDA/HDA (p=0.020). Among those who lived <10 km, all were compliant; whereas among those living >40 km, 44.4% were compliant. Patients living >40 km constituted 32 (41%) patients; out of which remission/LDA was seen in 7 (21.9%) and MDA/HDA was seen in 25 (78.1%). Association of distance from hospital >40 km with compliance (p=0.001) and with disease activity (p=0.012) was statistically significant. Out of 78 patients, 34 (43.6%) had education <10th std; out of which remission/LDA was seen in 4 (11.8%) & MDA/HDA in 30 (88.2%) patients (p<0.001). Eighteen (28.6%) patients had per capita income </= 4000 INR, of which none achieved target of remission/LDA and all had HDA/MDA (p<0.001). DI was calculated by Indian-HAQ score. 63% had DI<1, 16% had no disability and 20% had DI>1, none had DI>2. Correlation of disease activity score (DAS28-ESR) with DI was statistically significant (p=0.020) [Table 1] and [Table 2].
Table 1: Baseline characteristics

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Table 2: Factors affecting disease control

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Conclusion: Target of remission/LDA was achieved in 38.46% of patients. Average disease activity was 3.74 which was comparable to that from QUEST-RA study. Average DI was 0.49 (0 to 1.92). Significant social factors affecting disease activity adversely were compliance to treatment, use of alternative medicine, level of education, per capita income </= 4000 INR and staying far away from centre (poor access to rheumatology services). Correlation of disease activity score with DI also implies that those with low disease activity had less disability. Disease activity can be correlated with DI in our study because of short duration of disease.

Limitations: Since average disease duration is 5 years, it is less likely to get significant disability within such short period of time.


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Patient reported outcome measures in rheumatoid arthritis patients by using rheumatoid arthritis disease activity index-5 questionnaire and to correlate rheumatoid arthritis disease activity index-5 score with standard set of disease activity scores like (disease activity score-28 ESR and CRP), clinical disease activity index and simplified disease activity index

Bhavya Chintala, Lalit Duggal, Ved Chaturvedi, Neeraj Jain, Mayank Gupta; Department of Rheumatology and Clinical Immunology, Sir Gangaram Hospital, New Delhi, India

Background: Patient Reported Outcomes Measures (PROMs) refer to information that comes directly from patients. RADAI 5 (Rheumatoid Arthritis Disease Activity Index 5) is one of the most commonly used PROM which includes questionnaire on core set of symptoms in rheumatoid arthritis like pain, morning stiffness, and general wellbeing. Disease activity in RA is generally measured with standard set of criteria like Simplified Disease Activity Index (SDAI),Clinical Disease Activity Index (CDAI) and Disease Activity Score, DAS28 (ESR and CRP). The importance of this PROM is that one can assess the disease severity in patients view point, quickly, in busy OPD settings without any investigations. There is a paucity of information in Indian studies regarding correlation between PROMs and commonly used disease activity indices. So this study was conducted to emphasize the importance of PROMs and their relation to disease activity.

Objectives: The objectives was to study Patient Reported Outcome Measures in Rheumatoid Arthritis patients by using Rheumatoid Arthritis Disease Activity Index (RADAI 5) questionnaire and to correlate RADAI 5 score with standard set of disease activity scores like Disease Activity Score (DAS28 ESR and CRP), Clinical Disease Activity Index (CDAI) and Simplified Disease Activity Index (SDAI).

Methods: Patients with age more than 18 years and who were diagnosed as Rheumatoid Arthritis according to 2010 ACR- EULAR were included in the study. Patients should be diagnosed at least 6 months prior to enrolment into study. Pregnant patients and Patients who have severe anemia, hypothyroidism, and pulmonary, cardiac, liver, or renal disease were excluded from the study.

Results: A total of 50 patients were included in the study. The mean age of patients was 51.90±10.37 years and 82% of patients were females. The mean Tender joint count (TJC) and Swollen joint count (SJC) was 12.68 ± 6.21 and 9.52 ± 5.31 respectively. The mean DAS 28 ESR and CDAI was 5.82 ± 1.25 and 31.42 ± 16.10 respectively. The mean SDAI and RADAI5 SCORE was 33.08 ± 17.07 and 6.05 ± 1.47 respectively. The mean DAS 28 CRP was 5.22 ± 1.22. There was significant positive correlation of RADAI5 score with Tender joint count (r=0.70, p=0.0001) and Swollen joint count (r=0.71, p=0.0001). RADAI5 score was also significantly positively correlated with DAS 28 ESR (r=0.82, p=0.0001), CDAI (r=0.71, p=0.0001), SDAI (r=0.74, p=0.0001) and DAS 28 CRP [r=0.79, p=0.0001, [Table 1]].
Table 1

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Conclusion: PROM based disease activity measured by RADAI 5 score correlated well with composite disease activity indices like DAS28ESR, DAS 28CRP, CADI and SDAI. It also correlated well with tender joint and swollen joint count. As RADAI 5 score takes less time and more patient friendly, it can be used in busy clinical settings in place of composite indices for which laboratory parameters are needed and take relatively more time to calculate. In resource poor countries like India, RADAI 5 can be used even in primary care settings.


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Comparison of two doses of leucovorin in severe low-dose methotrexate toxicity: A randomized controlled trial

Varun Dhir, Mudit Bhargava, Chirag Rajkumar Kopp1, Shankar Naidu, Deba Prasad Dhibar, Atul Saroch, Alka Khadwal, Tarun Narang, Aman Sharma, Susheel Kumar, Shefali Sharma, Sanjay Jain; Department of Internal Medicine, Division of Rheumatology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Inadvertent overdose or renal failure may lead to severe hematological toxicity with low-dose MTX. An important antidote is leucovorin (folinic acid), however, its optimum dose is unclear.

Objectives: Compare two regimens of intravenous leucovorin - 15 mg or 25 mg every 6 hourly in severe low-dose methotrexate toxicity.

Methods: Open-label RCT that recruited patients with low-dose methotrexate (£ 50 mg per week) toxicity manifesting as severe cytopenia(s) (either WBC £ 2 x 10^9/L or platelet £ 50 x 10^9/L). They were randomized in a 1:1 ratio in to receive 15 mg or 25 mg q6h IV leucovorin. Allocation was concealed using sequentially numbered opaque envelopes. Kaplan-Meier analysis was used to determine difference in survival (censored at 30 days) and hematological recovery (WBC 3 4 x 10^9/L and platelet 3 100 x 10^9/L) and expressed in hazard ratios (95% confidence intervals).

Results: This study enrolled 38 patients with methotrexate toxicity who were randomized into two groups of 19 patients each. Most patients had underlying RA and had inadvertently overdosed. The median white blood count and platelet at presentation were 0.8 x 10^9/L and 23.5 x 10^9/L. The number (%) of patients who died over one-month was 8 (42) and 9 (47) in the 15 and 25 mg leucovorin groups respectively (Odds ratio 1.2, 95% CI 0.3 to 4.5, p=0.74). [Table 1] On Kaplan-Meier analysis, there was no significant difference in survival between the groups with hazard ratio of 1.1 [95% CI 0.4 to 2.9, p=0.84, [Figure 1]] On multivariable cox-regression, baseline serum albumin was the only significant predictor of survival, hazard ratio 0.3 (95% CI 0.1 to 0.9, p=0.02). The median time to hematological recovery in the two groups was 7 and 6 days, hazard ratio 1.0 (95% CI 0.4 to 2.7, p=0.9). Trial registration: CTRI/2019/09/021152.
Figure 1: Kaplan-Meier curves showing survival over 30 days in both groups after randomization. Lower panel: Odds ratio for death at 30 days between the two dosing strategies

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Table 1: Primary and secondary outcomes

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Conclusion: There was no significant difference in survival or time-to hematological recovery between the two doses of leucovorin. Severe low-dose methotrexate toxicity carried a significant mortality.


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Experience with tofacitinib in rheumatoid arthritis

Rahul Rudrakar, Kushagra Gupta, Ashok Kumar; Department of Rheumatology, Fortis Flt Lt Rajan Dhall Hospital, New Delhi, India

Background: Tofacitinib is the first oral Janus kinase inhibitor approved for the treatment of rheumatoid arthritis (RA). Since the expiry of its patent, the drug has become affordable to patients in India. The present study describes our experience with Tofacitinib in patients with RA.

Objectives: To study the 6-month outcome of patients with RA treated with tofacitinib.

Methods: We studied all consecutive patients with RA who received tofacitinib in the rheumatology clinic of our tertiary care hospital from December 2020 to December 2021. The drug was prescribed as per standard indications at a dose of 5 mg twice daily. Age, sex, duration of disease, prior treatment received, tender joint count, swollen joint count, patient as well as physician global assessment were recorded in a predesigned proforma. Lab investigations (CBC, ESR, CRP, LFT and KFT) were performed at baseline, 1 month 3 months and 6 months. Disease activity was assessed using SDAI at baseline and after 6 months. Adverse effects were carefully documented.

Results: 75 cases were recruited during the study period. Five patients were lost to follow up. Twelve patients stopped the drug after 3 months because of inefficacy/suboptimal benefit while 7 stopped the drug because of adverse effects. 51 patients out of 75 completed 6 months course of treatment and showed significant improvement (Low disease activity-23, remission-16). Adverse effects occurred in 27 (36%) patients and included weight gain (22%), cytopenia (18%), folliculitis (11%) and alopecia (11%). Others included anxiety, dyspepsia, constipation, chest pain, dyslipidemia, herpes zoster, hypertension, oral thrush, sensory neuropathy, somnolence and urinary tract infection.

Conclusion: Tofacitinib treatment resulted in remission in 16/75 (21%) and LDA in 23/75 (31%) in RA patients who were inadequate responders to methotrexate/biological drugs. Adverse effects were noted in 36%.


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An audit of 200 patients with rheumatoid arthritis attending the outpatient clinic of Department of Rheumatology, Manipal Hospital, Millers Road, Bengaluru

Veena Viswanath, B G Dharmanan; Department of Rheumatology, Manipal Hospitals, Bengaluru, Karnataka, India

Background: Patients with RA should be treated early and to a target of low disease activity state (LDAS) or remission. Though glucocorticoids are effective, because of their toxicity, the current emphasis is on the disadvantages than the benefits. A clinical audit of 200 consecutive patients with RA under our follow-up for a minimum of 6 months, was conducted.

Objectives: Determine remission rate, rate of steroid use and additional factors that influences disease activity.

Methods: Baseline characteristics were documented and DAS28 ESR assessed. Patients were categorized as those who met the target of remission or LDAS (Group A) and those with moderate / high disease activity (Group B). Variables of interest were tested using the Chi-square test, p-value calculated.

Results: Baseline characteristics of 200 patients are outlined in [Table 1]. Among the 197, in whom DAS28 scores were available [Table 2], 53% had achieved remission and 15.5% had LDAS (Group A); 20.5% and 9.5% had moderate and high disease activity (Group B). Steroid use was seen in 2.92% of the former and 40% of the latter. (p-value 0.001), suggesting, even addition of low dose steroids did not help attain target in a subset, probably because of a more aggressive phenotype. 29.93% achieved the target with monotherapy (Methotrexate in 75.61%). Fibromyalgia and affective spectrum disease was present in 8.76% and 3.65% of patients in Group A and in 31.67% and 16.67% of those in Group B and both were statistically significant. Prevalence of both was more in those with an inadequate disease control, because the PGA and hence DAS28 was higher in these subsets. Other comorbidities and choice of DMARDs did not show any statistically significant difference between the two groups.
Table 1: Baseline characteristics

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Table 2: Comparison of characteristics among Group A and B

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Conclusions: Newer treatments have considerably improved prognosis in RA, but many patients have a smouldering disease. Remission is possible without long-term steroids and high disease activity can persist despite use of low dose steroids.


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Institutional incidence of extra-articular manifestations of rheumatoid arthritis and its correlation with disease severity

Dipendra Singh Mandloi, Preksha Dwivedi; Department of Medicine, Gandhi Medical College and HH, Bhopal, Madhya Pradesh, India

Background: Rheumatoid arthritis is a systemic autoimmune disorder with extra-articular involvement. The prevalence of Ex-RA is reported to be 40% in a RA patient the population worldwide, they had to significant mortality. Ex-RA leads poor major diagnostic and therapeutic challenges. There is limited data regarding regarding the incidence of extra-articular manifestation in RA and its correlation with disease severity in the Indian population. Then this study was planned at tertiary care center.
Table 1: Comparison of elderly onset rheumatoid arthritis and young onset rheumatoid arthritis clinical characteristics

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Table 2: Comparison of baseline disease activity assessments at the time of recruitment

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Objectives: (1) To study the incidence of extra-articular manifestations of rheumatoid arthritis. (2) Correlation of extra-articular manifestations of rheumatoid arthritis with disease severity.

Methods: Study was conducted in the Department of Medicine, Gandhi Medical College and Hamidia Hospital Bhopal during July 2019 to June 2021. It was an Hospital based cross sectional observational study on 100 patients with rheumatoid arthritis, attending Hamidia Hospital, Bhopal. Locomotor system was examined and the total number of swollen and tender joints were counted and disease severity was calculated using disease activity score 28 (ESR). Statistical analysis was done using the IBM SPSS-20.

Results: About 90% of the cases were females in both case and control groups (p>0.05). Mean age of cases was 53.08 ± 9.9 years whereas that of controls was 41.6 ± 11.8 years. Methotrexte use was significant less in the subjects with EAM (P=0.04). Eye was the most common system involved in cases with extra-articular manifestations of rheumatoid arthritis 23 (46%), followed by pulmonary 13 (26%) and neurological manifestations 5 (10%).

Conclusion: Longer disease duration and female gender is associated with EAM. Secondary sjogren's is the most common EAM followed by pulmonary and neurological manifestation. Methotrexate use is associated with less EAM thus each patient rheumatoid arthritis should be treated early and aggressively.


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A comparison of elderly onset rheumatoid arthritis versus young onset rheumatoid arthritis in a tertiary care hospital in Karnataka

Shery Susan Philip, Ramya Sri Kodali, Ramya Janardhana, Sumithra Selvam1, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, 1Division of Epidemiology and Biostatistics, St. John's Research Institute, Bengaluru, Karnataka, India

Background: The impact of age on disease phenotype has been unclear in rheumatoid arthritis (RA) patients.

Objectives: To compare the clinical characteristics, comorbidities, and disease activity in EORA against YORA in a single-center RA cohort.

Methods: From 500 RA patients who participated in the Biotechnology Research Assistance Council-Clinical Trial Networks (BIRAC-CTN) registry at our center between March-November 2021, all consecutive EORA patients (disease onset>60 years of age) were identified. Among patients who had disease onset years of age (YORA), gender and disease duration matched controls were randomly selected in 1:2 ratio. Clinical characteristics like presenting symptoms, disease activity was documented and compared between both groups.

Results: The mean age of disease onset in EORA (n=27) was 64.1 ± 3.2 years and 41.6 ± 10.9 years YORA (n=54) respectively, majority being women (85%). Comparison of various clinical characteristics and disease activity between both groups are presented in [Table 1] and [Table 2]. Median early morning stiffness (EMS) duration was significantly higher in EORA [120 (60, 240) minutes] than YORA [60 (30, 75) minutes] (p=0.002). Both groups had predominantly symmetrical polyarticular arthritis at onset. Prevalence of type of joint involvement, pattern of arthritis and extra articular manifestations, did not differ significantly between 2 groups. Although statistically insignificant, median baseline swollen joint count (SJC28), and disease activity indices were noted to be higher in EORA. The patient and physician global assessments were comparable between the groups. Overall prevalence of comorbidities was higher in EORA compared to YORA. The prescription pattern was also similar in the groups.

Conclusions: In our study, except for duration of EMS which was significantly higher in EORA, other clinical parameters were comparable between EORA and YORA. These results suggest that age of disease onset does not seem to have a significant impact on disease characteristics in RA.


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Uric acid as marker of inflammation- the chicken or the egg conundrum

Aarushi, B K Kundu; Department of Medicine, ABVIMS and DR. RML Hospital, New Delhi, India

Background: Serum uric acid (SUA) has been associated with inflammation. UA may be a systemic inducer of inflammation and thus its role in systemic diseases as metabolic syndrome and atherosclerosis, as also in Systemic Lupus Erythematosus (SLE), though its role in Rheumatoid Arthritis (RA) is not well established. This study aims to estimate the prevalence of hyperuricemia in patients of RA in the Indian population and evaluate its role as a marker of inflammation.

Objectives: To estimate the prevalence of hyperuricemia in patients of RA and association of SUA levels with disease activity and already established inflammatory markers Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) in patients of RA.

Materials and Methods: One hundred and fifty patients of RA as per sample size calculation for prevalence study and fulfilling the inclusion and exclusion criteria were taken into the study and evaluated with respect to disease activity, current treatment, inflammatory markers ESR and CRP, and their SUA levels measured. A SUA level of 7 mg/dL or more was defined as hyperuricemia. Data was recorded in MS Excel sheet and statistical analysis done by JASP software.

Results: Nine of the one hundred and fifty patients (6%) were found to have SUA of 7 mg/dL or more. Both the hyperuricemic and normouricemic groups were similar in age, and gender. CRP, but not ESR, and swollen but not tender joint counts were significantly higher in the hyperuricemic group. SUA levels correlated positively with CRP levels, and with clinical disease activity index (CDAI). SUA levels did not differ significantly between patients positive for Rheumatoid Factor (RF) and/or Anti Citrullinated Cyclic Peptide (Anti-CCP) and those without, and between various treatment groups.

Conclusions: The prevalence of hyperuricemia in patients of RA was 6% in our study. Hyperuricemic patients of RA had higher levels of both CRP and CDAI. SUA levels did not differ with respect to seropositivity, or treatment regimens. Thus, SUA appears to be an independent marker of inflammation by itself. Further studies will be required to evaluate whether uric acid has a causal role in inflammation or is the effect of such.


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A study of interstitial lung disease in patients with rheumatoid arthritis

Nidhesh Khemchandani, Reshma Kaushik, Manju Saini; Department of Medicine, Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, India

Interstitial lung disease is the most common pulmonary manifestation of rheumatoid arthritis lung disease. Interstitial lung disease is associated with a high burden of morbidity and mortality.

Objectives: The present study was conducted to see the prevalence of ILD in patients of rheumatoid arthritis and to study the association between ILD and duration and clinical severity of rheumatoid arthritis.

Methods: 96 patients with a primary diagnosis of rheumatoid arthritis as per ACR/EULAR classification criteria were recruited and evaluated for severity of clinical disease activity by DAS 28 score, ESR, CRP and VAS score. All the patients were subjected to spirometry and HRCT chest for the assessment of interstitial lung disease.

Results: ILD was present in 16.7% rheumatoid arthritis patients [Table 1]. Majority of patients with ILD had more than 10 years of illness (43.8%) and high severity of disease (75%). Usual interstitial pneumonia type of ILD was seen in majority of RA patients (81.2 %). Among patients with ILD, spirometry pattern showed restrictive pattern in 68.8% patients. The association between duration of disease and ILD was not statistically significant (p value = 0.19) [Table 1]. The association between the severity of rheumatoid arthritis [Table 2] and interstitial lung disease was not found to be statistically significant (p value = 0.14) [Table 2]. The association between the spirometry pattern and interstitial lung disease was found to be statistically significant (p value = 0.001).
Table 1: Association of interstitial lung disease with duration of illness

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Table 2: Association of interstitial lung disease with severity of rheumatoid arthritis

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Conclusion: Interstitial lung disease was common in patients with rheumatoid arthritis (16.7% patients). The severity and duration of rheumatoid arthritis did not have any significant association with interstitial lung disease.


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Study of comorbidities and extra articular manifestations in patients of rheumatoid arthritis and their prevalence with seropositivity

O S Mahin, N R Shetty, Arjun S Das, Jyotsna Oak; Department of General Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

Background: Comorbidities of RA range from cardiovascular disease to malignancies. The spectrum of comorbidities include hypertension, diabetes mellitus, Osteoporosis, Hypothyroidism, Cardio vascular diseases (ischemic heart disease, stroke), Dyslipidemia, Peptic ulcer disease, Chronic liver disease, Bronchial asthma, malignancy.

Objectives: (1) To study the co morbidities in a patient of rheumatoid arthritis and their prevalence with Rheumatoid factor and Anti CCP positive patients. (2) To observe the extra articular manifestations in a patient of rheumatoid arthritis and their prevalence in seropositive and seronegative patients. (3) To study the relation between severity of disease by DAS 28-ESR score and extra articular manifestations.

Methods: Patients of Rheumatoid arthritis of either sex attending the Rheumatology Out Patient Department were chosen. Patients aged above 18 years of either sex, fulfilling the 2010 ACR/EULAR criteria for the diagnosis of Rheumatoid arthritis were enrolled. Rheumatoid factor, Anti CCP, CBC, ESR, CRP, Thyroid profile, Vitamin D and B12 level, FBS, PLBS, ANA by IF, Urine Routine, ECG, Chest X-ray, 2 D echo were collected. Joint assessment was done by counting the number of tender and swollen joints and calculation of DAS 28 ESR score. Past history of Coronary artery disease, Diabetes mellitus, Hypertension, Hypothyroidism on treatment were noted.

Results: Among 217 patients, 89.4% were females. The mean age was 54 years and mean duration of disease was 9.5 years. Among 92.1% of seropositive patients, 89.2% were rheumatoid factor positive and 54.4% were anti CCP antibody positive. The most common comorbidity was Vitamin D deficiency (51.2%). Other comorbidities were DM (18%), Hypertension (35%), Hypothyroidism (38.7%), CAD (10.6%), Vitamin B12 deficiency (18.4%), Osteoporosis (10.6%) and Osteoarthritis (16.1%). The most common extra articular manifestation in this study was Anaemia (50.7%). Other extra articular manifestations included ILD (9.2%), Vasculitis (5.1%), Secondary Sjogren's syndrome (9.2%) and Rheumatoid nodules (1.4%). There is a significant association between the EAMs and disease activity (DAS28-ESR score) (p-value 0.000).

Conclusions: Among comorbidities, Vitamin D deficiency and Anemia are the most prevalent manifestation. Presence of extra articular manifestations increases the severity of disease in rheumatoid arthritis patients.


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Knowledge about rheumatoid arthritis among 3rd, 4th and final year MBBS students of a medical college in South India

D Marwaha, N Ajithkumar, C V Sreelakshmi1, P Dhevarsh1, V Marwaha1; Department of Commerce and Management, Amrita School of Arts and Sciences, Amrita Vishwa Vidyapeetham, 1Department of Clinical Immunology and Rheumatology, Amrita School of Medicine, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

Background: Rheumatoid arthritis (RA) affects approximately 1% of the worldwide population. More than 55% of India's vast population is rural, where primary health care centres (PHC) are the principal point of contact. A PHC includes two medical officers (MBBS and AYUSH) at the centre. The PHC acts as a referral unit for higher medical centres. Considering the burden of RA, MBBS students should be adequately trained to assess, diagnose, treat and refer RA patients.

Objectives: To assess the knowledge of Rheumatoid Arthritis in 3rd, 4th and final year MBBS students of a medical college in southern India.

Methods: A cross-sectional study was conducted online and offline by distributing a self-structured validated questionnaire. It assessed basic knowledge of RA in 3rd, 4th and final year students in two domains – (i) Clinical features and epidemiology, and (ii) Radiological features and treatment. The final multivariate analysis was done using SPSS software (version 23).

Results: 142 students participated [Table 1], 3rd year (n=49), 4th year (n=50) and final year (n=43). There were 69% females and 31% males [Table 1]. The mean self-assessment value was 4.82 ± 1.944 and the mean value scored in the questionnaire was 7.27 ± 2.183. 60% of students scored lower than the mean score for domain A. Significant association [Table 2] was observed between the year of study and final score (0.252) [Table 2]. Year of study and self-rated knowledge was also significantly associated (0.223).
Table 1: Characteristics observed based on gender

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Table 2: Significant associations between variables

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Conclusions: Students have lower confidence while assessing themselves in comparison to their actual score. There is a gap in the knowledge quotient of students with respect to clinical features and epidemiology. This needs to be addressed. Similar studies in the country will help elucidate these gaps. These challenges must be kept in mind while revising the medical curriculum.


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Tofacitinib: Real life OPD use and follow up data

S Pandya, R Solanki, T Parikh; Department of Rheumatology, Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: While tofacitinib has had a very good and early response in patients of RA, we observed a waning effect over time and wanted to study this.

Objective: In our earlier presented data, about half of our patients had a EULAR good response at one month of tofacitinib. We wanted to evaluate response of RA patients to tofacitinib on longer follow up in our patients to see if the efficacy was sustained.

Methods: Retrospective analysis of Data collected from December 2020 to August 2022 was done. Descriptive statistics were applied and response in terms of EULAR good response (DAS 28 CRP) at last follow up was measured. Informed consent was taken.

Conclusions: The remarkable efficacy seen in patients started on tofacitinib at 1 month was not sustained at 11 months follow up. More follow up data is needed to validate the same.


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Response to rituximab in rheumatoid arthritis cases responding inadequately to synthetic DMARDs

Manali Chandra, Kaushik Basu; Medical College, Kolkata, West Bengal, India

Background: Rheumatoid Arthritis (RA) is a long known debilitating systemic disease chiefly affecting joints. Excellent synthetic and biologic DMARDs are available to target the same, one of them being the anti CD20 monoclonal antibody, Rituximab. Although studies suggesting effectiveness of Rituximab on RA are there, time to earliest and maximum response were not known.

Methods: This tertiary care centre based observational longitudinal study involving 34 patients of RA with inadequate response to sDMARDs who were receiving Rituximab 1000 mg two doses 2 weeks apart were observed and followed up from the time of drug initiation every month for 6 months to assess disease activity reduction in terms of DAS 28-CRP, CDAI, SDAI with objectives of finding time to (a) First response (b) Maximum response (c) First flare occurring after Rituximab injection.
Table 1: Post hoc test showing significance of pairwise difference of mean disease activity scores 28 from the start of study to 6 months of follow up

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Results: All patients had obtained response as per DAS28-CRP; 50% population has achieved response as per DAS 28 -CRP as early as between 1st and 2nd month, not seen in any other study till now, and by 3 months 99% of study population had responded. Month in which disease activity has reached its lowest in terms of DAS 28-CRP and CDAI is between 4th and 5th month, between 5th and 6th month in terms of SDAI. No patient went into relapse as per DAS 28-CRP in the 6months of follow up period [Figure 1].
Figure 1: Line diagram showing mean disease activity as per DAS-28, CDAI and SDAI from time of first contact to over the subsequent 6 months

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Conclusion: Response to Rituximab may be attained as early as within one month, faster than most DMARDs with maximum response in 4th-6th month depending on disease activity scoring used. Remission sustained for the 6 months of follow up. Further controlled trial is needed on this.


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Assessment of pain by rheumatoid arthritis pain scale and its correlation with disease activity in patients of rheumatoid arthritis

Harshil C Shah, Rajneesh Singh; Department of Medicine, ABVIMS and Dr. RML Hospital, New Delhi, India

Background: Rheumatoid Arthritis Pain Scale was developed as a self-reporting instrument covering all dimensions of pain in RA including cognitive aspects, behavioral changes, sensory phenomenon and psychological disturbances as a qualitative single score. However, there is paucity of data on RAPS and studies on its utility as an assessment tool in RA patients for disease activity or remission are scarce.

Objectives: To find correlation of Rheumatoid Arthritis Pain Scale (RAPS) score with disease activity.

Methods: This cross-sectional observational study conducted between 1st January, 2021 and 31st May, 2022 in a tertiary care hospital in New Delhi enrolled 40 patients with RA. All patients were asked to complete the RAPS questionnaire. Blood investigations like ESR and CRP were done. Disease severity was assessed by tender joint count, swollen joint count and DAS28 score, CDAI score, PGA, EGA and pain-VAS score were calculated. were calculated. Spearman Correlation coefficient was used for correlation of RAPS score with various parameters. P value of less than 0.05 was considered significant.

Results: The mean value of RAPS score was 50.22 ± 18.6. Positive correlation was seen between RAPS and DAS28 as well as CDAI with correlation coefficient of 0.901 and 0.875 respectively. No correlation was seen between RAPS score and DAS28 ESR as well as CDAI in remission stage. (correlation coefficient 0.037 and 0.067 respectively). Significant correlation was found between RAPS score and TJC, SJC, PGA, EGA, Pain-VAS, ESR with correlation coefficient of 0.835, 0.608, 0.881, 0.848, 0.887, 0.859 respectively (p<0.0001)

Conclusion: RAPSa has a strong correlation with established disease activity measurement tools suh as DAS28 and CDAI, and with other disease activity parameters such as SJC, TJC, PGA, EGA, Pain-VAS and ESR and can be used as a reliable and clinically valuable measure to assess pain and disease in RA patients.


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Safety and efficacy of tofacitinib in autoimmune rheumatic diseases: A retrospective study from a rheumatology clinic in Dehradun, North India

Kamal Bhatt; Prerna Clinic, Dehradun, Uttarakhand, India

Background: Tofacitinib has emerged as an important drug for the management of RA and other rheumatic diseases. But there is ongoing concerns regarding its safety in long term use.

Objective: This retrospective study was undertaken with an aim to analyze the adverse effects of Tofacitinib in RA and other rheumatic diseases and to assess the efficacy in patients of RA.

Methods: Records of all patients advised tofacitinib between December 2020 and August 2022 were analyzed. Clinical parameters and adverse effects were noted for all patients, while efficacy was noted only for patients of RA. This was done by DAS scores whenever available or by clinical assessment.

Results: Total number of patients was 154. 131 (85%) were females, mean age was 45.66 years and mean follow up was 8.79 months. 132 patients (85.7 %) had RA, 15 had SPA, 4 had JIA, 2 had Psoriatic Arthritis and one Uveitis. 35 patients (22.72 %) reported adverse effects (AE). One patient died due to suspected Pulmonary Embolism. One patient had a non-fatal Acute MI and another required hospitalization because of severe pneumonia. Other AEs include abdominal pain /dyspepsia (9), leukopenia (5), deranged LFT (3), skin allergy/urticaria (2), anorexia (2), pneumonia (1-responding to oral antibiotic), uncontrolled hypertension (1), severe dermatophytosis (1) Herpes Zoster (1), and miscellaneous (7). No patient developed tuberculosis. Among 132 patients of RA, twenty-six patients (19.69 %) achieved remission, 77 (58.33%) had some effect and the rest 29 (21.96 %) had no benefit from the drug.

Conclusions: There were three major adverse events among 154 patients, including one death, and 32 minor adverse effects. This emphasizes the need for appropriate patient selection and constant surveillance for its safe use. Tofacitinib appears to an effective drug for the management of RA with almost two-third of patients benefitting from the treatment.


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Prevalence of comorbidities in rheumatoid arthritis: A single centre tertiary health care hospital cross-sectional study

Smruti Ramteke, Sanjay Ramteke, Jayashri Shembalkar, Sandeep Yadav; Department of Rheumatology, Jasleen Hospital, 1Department of Neurology, Government Super Speciality Hospital, 2Department of Rheumatology, Getwell Hospital and Research Institute, Nagpur, 3P. D. Hinduja National Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Background: Patients with rheumatoid arthritis (RA) are at increased risk of developing comorbid conditions, affecting overall treatment outcomes.

Objectives: To evaluate the prevalence of various comorbidities in patients of rheumatoid arthritis patients.

Study Design: A cross-sectional analysis was performed on all consecutive Rheumatoid Arthritis (RA) patients meeting the 2010 ACR criteria who visited the outpatient clinic of a tertiary healthcare facility between 2020 and 2021. Interviews and the verification of charts and reports found in the patient records were used by trained clinical research associates to collect the data in accordance with a pre-structured pro forma. The research was done on the following comorbidities: Diabetes, hypertension, and hypothyroidism.

Results: This was a single-centre study, and patients with incomplete data were excluded. The number of subjects considered for the analysis was 1000. The mean age was 47.68 ± 11.46 years. The total number of females were 880 and male were 120, and the male-to-female ratio was 1:7. Hypertension was the most common comorbidity seen in 109 (11%) patients, followed by hypothyroidism in 85 (8.5%) patients. Diabetes was prevalent in 7% (70) of patients. Ischemic heart disease was seen only in one patient and cancer in 2 patients [Table 1]. 6.8% of females in the age group less than 45 reached menopause. Sub-group age analysis showed that the prevalence of hypothyroidism was similar across all subgroups, with 7-10% of patients having hypothyroidism, a similar prevalence reported by other Indian studies. Prevalence of diabetes was similar to COMORA study internationally and studies from India. Prevalence of HTN was lower in the current study subjects compared to similar studies reported from other parts of the world and India.
Table 1: Prevalence of different comorbidities in rheumatoid arthritis patients

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Table 2: Prevalence of comorbidities according to various age sub group

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Conclusion: A significant proportion of patients with rheumatoid arthritis have comorbidities, with hypertension being the most prevalent. The prevalence of diabetes and hypothyroidism was consistent with similar studies from India and internationally but was lower for hypertension.


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Assessment of cardiovascular risk prevalence, to look for subclinical atherosclerosis by measuring CIMT using carotid Doppler

Tamanna Singh, Manaswi Chaubey, Jaya Chakravarty, Madhukar Rai, Ranjan Bhatnagar, Sumeet Chatterjee; Department of General Medicine, Sir SunderLal Hospital, BHU, Varanasi, UP

Background: Rheumatoid arthritis (RA) is a chronic, inflammatory, autoimmune disorder which attacks multiple joints, skin, eyes, lungs, heart and blood vessels. Its worldwide prevalence is 1.1% and in India it is 0.5-0.75%. Female gender, increased age, smoking, obesity are some of the important risk factors. The most common cause of mortality are ischemic heart diseases. With this study, we will assess the CVS risk in RA patients.

Objectives: To study prevalence of subclinical atherosclerosis in RA by using carotid intima media thickness.

Methodology: A case control cross sectional study done in the Department of General Medicine, SSH BHU. The study will include 50 RA patients and 50 controls without any co morbidity. Once the diagnosis of rheumatoid arthritis is confirmed, evaluate the CVS risk in RA patients, by measuring CIMT (carotid intima media thickness) using carotid Doppler using cut off of 0.9 mm to look for subclinical atherosclerosis.

Results: Patients with RA had a higher CIMT (mm) compared with healthy controls (LIMT: 0.604 ± 0.1340 vs. 0.388 ± 0.0895; RIMT: 0.6010 ± 0.13795 vs. 0.3890 ± 0.07908) with a P value < 0.001 showed significant results as compared to the controls.

Conclusion: Subclinical atherosclerosis (increase CIMT) is common in RA patients as compared to controls, which can be used as a surrogate marker to look for the cardiovascular risk in RA patients.


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The clinical spectrum and therapeutic management of macrophage activation syndrome associated with systemic lupus erythematosus: A single-center retrospective study

Mavidi Sunil Kumar, Soumya Dey, Dipendra Ghosh, Hiramanik Sit, Geetabali Sircar, Parasar Ghosh; Department of Clinical Immunology and Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Macrophage Activation Syndrome (MAS) is a fatal complication of hyperinflammation that is seen in patients with Systemic Lupus Erythematosus. Literature regarding the MAS in SLE has been relatively scarce.

Objectives: To describe the clinical, laboratory characteristics, precipitating factors, and treatment outcomes in a cohort of patients with SLE-associated MAS.

Methods: We retrospectively analyzed the clinical, laboratory, precipitating factors and treatment outcomes of 33 patients with SLE-associated MAS diagnosed between January 2020 and July 2022. Categorical variables were presented in the form of frequency and percentage, quantitative variables were presented in the form of mean ± standard deviation.

Results: The mean age at the onset of MAS were 22.45 years (± 10.89). The mean disease duration was 38 months. The average hospital stay was 18 (± 8) days. Eighty percent of our patients were female. Sixty-six percent (n=22) of the MAS episodes were secondary to disease activity and thirty-four percent (n=11) were secondary to infections, predominantly lower respiratory tract infections. The median SLEDAI score was 15.87 (8-26). The majority of patients had fever, splenomegaly, cytopenias, elevated triglycerides, transaminitis, hyperferritinemia, and elevated LDH levels. Nine patients underwent bone marrow study. Four out of nine patients had bone marrow pictures suggestive of hemophagocytosis. Glucocorticoids were used in all patients. In patients with high disease activity without evidence of infection, methylprednisolone pulse therapy was given for 3-5 days (mean dose 1.325 g followed by oral prednisolone at 1 mg/kg/day). Six patients required IVIG. Ninety-three percent (n=31) of patients responded to therapy and 2 patients died.

Conclusion: SLE-associated MAS usually occurs in young females majorly, secondary to active disease followed by infections. Bone marrow hemophagocytosis was seen in half of the patients. SLE-associated MAS had a good short-term prognosis.


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Lupus nephritis post COVID-19 vaccination: Waking a sleeping giant?

Pallavi Vij, Uma Kumar; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Background: COVID-19 vaccine associated adverse effects are mostly mild to moderate and largely transient in nature. However, reported cases of autoimmune diseases in close temporal association with vaccination have hinted towards a possible autoimmune dysregulation induced by the vaccines.

Case Report: We report a case of a 20-year-old female presenting with progressive anasarca and bullous skin lesions within 2 weeks of receiving her first dose of COVID-19 vaccine. Her routine investigations revealed nephrotic range proteinuria (2.1 g/day) with active urinary sediments, deranged renal parameters, hypoalbuminemia, hypocomplementemia, positive ANA and spiking of anti-dsDNA levels. On renal biopsy membrano-proliferative pattern with full house DIF consistent with lupus nephritis was seen [Figure 1]. The skin lesions were diagnosed as linear IgG/IgA disease on biopsy [Figure 2]. She was started on oral steroids and MMF. After an initial increase in the serial 24-h urine protein (from 12 g/day to 22 g/day) the proteinuria responded to treatment with improvement in renal function. However, in view of treatment unresponsive anasarca and skin lesions despite MMF 3 g/day for nearly 3 months, the patient was started on injection cyclophosphamide as per NIH protocol. The patient achieved remission with fall in proteinuria after initial 3 pulses of injection cyclophosphamide and is planned to be on maintenance therapy with MMF after completion of induction phase.
Figure 1: Renal biopsy of the patient showing Class VI lupus nephritis with an activity index of 8/24 and a chronicity index of 1/12

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Figure 2: Bullous fluid filled skin lesions diagnosed as linear IgG/IgA disease

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Conclusion: This case highlights the possible role of COVID-19 vaccines in triggering autoimmune response especially in people with risk factors for developing autoimmune diseases. A high level of awareness among physicians is critical for early recognition and prompt treatment.


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Utility of sCD14 and IL-6 in differentiating flare and infection in a febrile patient with systemic lupus erythematosus

Kishan Majithiya, Komal Singh, Pankti Mehta, K Chengappa1, Able Lawrence, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, SGPGIMS, Lucknow, Uttar Pradesh, 1Department of Clinical Immunology, JIPMER, Puducherry, India

Background: Evaluation of patients with SLE presenting with fever is a major diagnostic challenge. A combination of biomarkers have been assessed previously to accurately predict infection or flare with a modest accuracy.

Objectives: To study utility of IL-6 and sCD14 in addition to traditional biomarkers to differentiate flare from infection in SLE and to validate previously proposed composite scores.

Methods: Febrile patients with SLE were evaluated and routine investigations were carried out. Additionally, they were tested for NEUT-x, y, z indices,[1] neutrophil to lymphocyte ratio (NLR),C3, C4, anti-dsDNA, ESR, CRP, procalcitonin, IL-6 and sCD14. The febrile episode was classified as infection, flare or both. Univariate and multivariate analyses were carried out using GraphPad version 9.4.1 and two previously proposed calculators were also validated. [1,2]

Results: Of 134 febrile episodes in 128 patients with SLE, 38 were due to infection, 50 were due to flare, 47 were due to both. Those with both flare and infection (n=47), and with mycobacterial infection (n=1) were excluded from further analysis. High SLEDAI2K (p=0.0001), anti-dsDNA (p=0.0003), ESR/CRP ratio (0.0023) and low C3/C4 (<0.0001) were characteristic of flare. High TLC (p=0.0065), NLR (p=0.0239), NEUT-x (p=0.001), -z (p=0.0014), CRP (p=0.0023) and PCT (p=0.03), sCD14 (0.0069) were seen with infection [Table 1]. Using Multiple logistic regression model we found TLC, anti-dsDNA antibody and C4 level were able to differentiate SLE flare from infection. The previous suggested models with 3-5 low cost variables performed well with AUC varying between 0.75-0.87. Addition of IL6 levels and/or sCD14 levels led to only minor increase in AUC in these models [Figure 1].
Figure 1: Predictive value of composite scores

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Table 1: Comparative analysis of variables between those with infection and disease activity

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Conclusions: sCD14 is a good marker of infection. Previously suggested models perform well and addition of IL-6 and sCD14 leads only to a minor improvement in the prediction of infection in a febrile SLE patients.


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Short term outcome in NPSLE following treatment with six doses of cyclophosphamide

Deepti Joy, P S Arul Raja Murugan, R Ramesh, S Mythili; Department of Clinical Immunology and Rheumatology, Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Background: Neuropsychiatric SLE remains the most challenging manifestation of SLE in terms of studying disease pattern, outcome and response to treatment in view of the heterogeneity of the manifestations.

Objectives: To study the profile and short-term outcomes of NPSLE patients who have received 6 doses of cyclophosphamide for their disease.

Study Methods: Review of case records of NPSLE patients treated with 6 doses of cyclophosphamide. Univariate and multi variate analysis by nonparametric tests to evaluate for associations.

Results: Twenty cases of NPSLE were included [Table 1]. No patients had recurrence of seizures after treatment. Three patients with peripheral neuropathic symptoms had partial improvement. SLEDAI at baseline compared to SLEDAI after 6 doses using Wilcoxon signed rank test Z -3.92 (p<0.001). The mean steroid dose at onset versus at the end of 6 months was 36.9 mg versus 8.3 mg (Z-3.93, p<0.001). The spearman's rho for disease duration with SLEDAI after 6 doses and that of NPSLE duration with SLEDAI after doses was 0.14 (p0.56) and -.24 (p 0.31) respectively. Mann Whitney U for patient SLEDAI after treatment with occurrence of infection was 39.5 (p 0.767), mean rank for those without infection was 10.68 versus 10.08 for those with infections. There were no deaths in the studied group of patients.
Table 1: Baseline

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Conclusion: All patients having seizures as part of NPSLE had complete resolution. Those with peripheral nervous involvement had partial improvement in deficits. Cyclophosphamide brought down the disease activity and need for steroids after treatment with 6 doses. Even though 6 patients had infections during the total course of treatment, there were no deaths.


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Latent tuberculosis infection and its associations with clinical and serological parameters in systemic lupus erythematosus

M S Gayathri, Chengappa Kavadichanda, G Aishwarya, D Bairwa, M M Thabah, Sonali Sarkar1, Prakash Babu Narasimhan; Departments of Clinical Immunology and 1Preventive and Social Medicine, JIPMER, Puducherry, India

Background: Latent tuberculosis infection (L-TB) detected by mycobacterium tuberculosis (TB) IFN-γ release assay (TB-IGRA), besides identifying latent infection may predict the disease status in SLE.

Objectives: This study aimed to assess prevalence of L-TB and its association with clinical and serological parameters in SLE.

Methods: This is a single center cross sectional study. Adults with SLE with no past h/o TB were recruited (n=219). Demography, disease activity indices, autoantibody profile and steroid use were noted. Presence of Latent TB was assessed in all patients using TB-IGRA. SLE cases were divided into 2 groups-IGRA positive and IGRA negative. The collected parameters were compared between the 2 groups.

Results: Among 219 patients [Table 1], 41 had latent TB resulting in a point prevalence of 18.7 %. Among antibody profile, proportion of Ro 52 was higher in IGRA positive group (43.9%) than IGRA negative group (25.3%) (p=0.014) and proportion aCLA IgM antibody higher in IGRA negative group (12.9%) than positive group (2.4%) (p=0.032) [Table 1]. Comparison of clinical features yielded no statistically significant difference in the 2 groups. Serology revealed greater proportion of low C4 in IGRA negative as compared to positive group (p=0.036). There was no correlation of SLE disease activity with IFN gamma levels in the unstimulated tube of TB-IGRA.
Table 1: Comparison of clinical and laboratory parameters between interferon gamma response assay positive and interferon gamma response assay negative systemic lupus erythematosus cases

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Conclusion: Prevalence of latent TB in SLE cases was 18.7% which is lesser than latent TB prevalence in the general population (40%). Higher association of anti-Ro 52 antibody in IGRA positive group along with higher aCLA IgM and low C4 in IGRA negative group were found, whose physiological significance is unknown. IFN gamma levels in unstimulated tube of TB-IGRA did not seem to correlate with SLE disease activity. Further studies would be required to throw more light on the interplay between latent TB and SLE.


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Long term renal outcome of membranous lupus nephritis: A single centre bidirectional observational study from South India

Sowmya Kotha, D Phani Kumar, Meghana Gawali, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Pure membranous LN (MLN) accounts for 10–20% of total cases of LN. Risk of ESRD in LN is ranging from 0 to 23% at 10 years. There is limited data available on long term renal outcomes for pure MLN.

Objectives: To study long term renal outcomes in MLN (minimum follow up period after renal biopsy is 2 years).

Methods: We studied 30 patients of MLN diagnosed between January 2013 to December 2020 [Table 1]. Clinical data (Demographic data, serum creatinine, eGFR, complete urine examination and 24 hr urinary protein or spot urine protein creatinine ratio [SPCR], immunosuppression data) was obtained from medical records at the time of nephritis onset and during follow up visits (between January 1 to August 31, 2022). Renal outcomes were determined in terms of complete renal response (CRR), renal relapses, refractory nephritis, ESRD, Renal replacement therapy (RRT), and death. Descriptive statistics were used accordingly.
Table 1: Baseline demographic, clinical, laboratory and treatment data

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Results: Median duration of follow up is 5 (IQR-4-7) years. Mean (SD) spot urine protein creatinine ratio at onset of nephritis was 2.1 (1.3) with eGFR of 119 (26) ml/min/1.73 m2 [Table 1]. Initial induction therapy used was cyclophosphamide in 50% of patients and mycophenolic acid in 50%. CRR was achieved in 22/30 (73%) in a median duration of 10 (IQR-6-12) months, switch to other immunosuppressive therapy was needed in 10/30 (33.3%). In a subgroup analysis, induction therapy with MPA and CYC found equally effective for attaining CRR (p value-0.68). At 5 year median follow up mean SPCR was 0.7 (1.18) with mean eGFR of 118 (27) ml/min/1.73 m2. Five patients relapsed and five developed refractory lupus nephritis. None of the patients developed ESRD or required RRT in follow-up. Death was seen in 2 patients, with cause being rapidly progressive renal failure with sepsis.

Conclusion: In our cohort of MLN, complete renal response is attained in most irrespective of induction therapy with good renal survival in follow up. Renal relapse and refractory nephritis seen in less than one fourth of patients.


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Safety and efficacy of initial prednisolone dose for induction of renal remission in lupus nephritis patients presenting with reduced renal function

Gautam Raj Panjabi, Parasar Ghosh, Atanu Pal1; Departments of Clinical Immunology and Rheumatology and 1Nephrology, IPGMER, Kolkata, West Bengal, India

Background: Lupus nephritis (LN) is a common cause of renal impairment, morbidity and mortality in systemic lupus erythematosus (SLE) patients. LN complicated with renal impairment is a common complication and is reported variably in different studies whose management includes high dose glucocorticoids and immunosuppressive agents. However controlled studies with respect to glucocorticoid dosage are limited. Moreover, there is increase in risk of infections and related mortality that correlate with dose and duration of corticosteroid therapy.[1] We conducted this study with an aim to compare the efficacy and safety of the two different glucocorticoids doses in LN patients with renal impairment.

Objectives: The in-between group differences in – (1) Renal response (complete remission, partial remission, treatment failure, End stage renal disease (ESRD) or death) at 12 months from onset of insult. (2) Rate of infections and related mortality.

Methods: We enrolled 27 patients of SLE presenting with nephritis and renal impairment as defined by KDIGO criteria for Acute Kidney disease (AKD)[2] from January - December 2021 hospitalized in our centre [Table 1]. Following intravenous methylprednisolone 250 mg-1000 mg for upto three days, patients were randomly assigned to receive oral prednisolone either at 1 or at 0.5 mg/kg body wt with tapering as per protocol in addition to standard of care.
Table 1: Baseline demographic and clinical characteristics of systemic lupus erythematosus patients with acute kidney disease

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Results: Majority were females (n=25). The mean age was 27 (± 8.5 SD) years. The mean disease duration was 59 months. Of 27 patients 19, 8 patients respectively were randomized to 1 mg, 0.5 mg/kg body wt. 68.4% (n=13/19), 75% (n=6/8) achieved renal remission (CRR/PRR), 10.5% (n=2/19), 25% (n=2/8) developed ESRD, 21% (n=4/19), 25% (n=2/8) had treatment failure, 21% (n=4/19), 25% (n=2/8) died in 1mg, 0.5 mg/kg body wt groups respectively [Figure 1].
Figure 1: Primary outcome of systemic lupus erythematosus patients with acute kidney disease following treatment at 12 months from onset

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Conclusions: Proportion of patients with renal remission were almost comparable. However, the group receiving 1 mg/kg body weight prednisolone did better with respect to treatment failure, progression to ESRD (morbidity) and mortality.


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Prevalence and associations of myositis in an Indian inception cohort of lupus

Liza Rajasekhar, Vineetha Shobha1, Meenakshi Ponnana, Chengappa Kavadichanda2, Ashish Jacob Mathew3, Saumya Ranjan Tripathy4, Manish Rathi5, Parasar Ghosh6, Ranjan Gupta7, Avinash Jain8, Amita Aggarwal9; Department of Clinical Immunology and Rheumatology, Nizam Institute of Medical Sciences, Hyderabad, Telangana, 1Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, Karnataka, 2Department of Clinical Immunology and Rheumatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 3Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, 4Department of Rheumatology, SCB Medical College, Cuttack, Odisha, 5Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, 6Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, 7Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, 8Department of Clinical Immunology and Rheumatology, SMS Medical College and Hospital, Jaipur, Rajasthan, 9Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Muscle inflammation in systemic lupus erythematosus (SLE) presents as generalised myalgia with active disease elsewhere. There is data paucity on lupus myositis.

Objectives: To assess the myositis frequency and association in an inception cohort of SLE from India (INSPIRE) at 10 tertiary care hospitals.

Methods: The INSPIRE database includes SLE patients satisfying SLICC classification criteria with disease duration <3 years. Myositis was defined by proximal muscle aching/weakness, associated with elevated CPK/LDH/AST/ALT. Other causes/drug induced myopathy/muscle enzyme elevation were excluded. Demographics, lab features, autoantibodies, disease activity (SLEDAI, BILAG score), damage (SDI) and mortality were retrieved from the database. SLE myositis patients (cases) were compared with SLE patients (controls). Student's t-test was used for mean difference between groups and chi-square test for difference in proportions. Presence of infection, BILAG A/B, myositis and gender were used as mortality predictors using binomial logistic regression.

Results: A total of 2210 patients were enrolled in the study with mean (SD) age at diagnosis, 28.9 ± 0.79 years; disease duration, 16.4 ± 0.45 months. Comorbidities included hypothyroidism (16.4%), hypertension (8.8%), diabetes mellitus (1.6%). 133 patients died during mean follow-up of 22.4 ± 12.5 months. 125 (5.6%) patients had myositis, with myalgia (88%). On univariate analysis, cases were younger, with frequent fever, ACLE, oral ulcers, PE, leukopenia, thrombocytopenia, lower haemoglobin, complements, albumin and higher ALT, AST, LDH, CPK, SLEDAI and BILAG scores than controls [Table 1]. Ro60 antibodies were frequent in cases. Twelve cases had muscle damage as assessed by SDI. Cases had a significantly lower survival (87% vs. 94%, p<0.001 [Figure 1]). Univariate analysis identified fever, SLEDAI, CPK as myositis predictors (p<0.05). Binomial logistic regression identified infection as mortality predictor (p<0.03).
Figure 1: Survival in patients with myositis

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Table 1: Comparison of clinical and laboratory features in cases and controls

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Conclusion: Myositis prevalence with early, very active disease was 5.6 %. Cases had more active disease elsewhere, had similar damage but lower survival than controls. Myositis was not a predictor of mortality.


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To differentiate flare and infection in lupus patients using validated calculator in a tertiary center of Northern India

Mukesh Kumar Maurya, Abilash V Krishnan, Nishant G Kamble, P Ankush, Mukesh K Maurya, Pankti Mehta, T G Sundaram, Puneet Kumar, Urmila Dhakad; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Patients with SLE are more prone for infections owing to disease activity, abnormal immune function, and long term immunosuppressive agents. Bacterial infections are the major cause of morbidity and mortality. Differentiating bacterial infections from flare (disease activity) is a diagnostic challenge.

Objectives: To differentiate infections from flares (disease activity) in patients of SLE.

Methods: Patients (n-53) with SLE fulfilling ACR/EULAR 2019 criteria presenting with suspected infections or flares between July 2021 to July 2022 were included in the study. TLC, ESR, CRP, C3, C4, anti-ds DNA, and procalcitonin were tested in addition to routine investigations. The febrile episodes were classified to three groups based on clinical and laboratory assessment, infections, disease flares or both. Patients with both infections and flares were excluded from the study. Statistical analysis was done using GraphPad prism v8.4.2 and SPSS version 23, with p values < 0.05 considered to be significant. A previously validated model by Pankti et al. (Differentiating flare and infection in febrile lupus patients: Derivation and validation of a calculator for resource constrained settings) was used (Reference attached). The performance of a above devised binary logistic regression model using Age, TLC and CRP was evaluated. A composite score was calculated with a calculator incorporated in the spreadsheet.







Results: Out of 53 patients infections were present in 15 (28.3 %) patients and disease activity in 38 (71.6%). Most common infection was UTI followed by LRTI. Using the above model, an AUC of 0.63 (0.5256 to 0.7386) with p value of 0.0191 with sensitivity of 71.70% (58.43% to 82.03%) and specificity of 54.72% (41.45% to 67.34%). A model by Pankti et al. using age, TLC, and CRP using 80% of the patient cohort with an AUC of 0.88 (0.78- 0.97) which was validated in remaining 20% to have an AUC of 0.74.
Table 1: Causes of elevated serum creatinine

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Conclusions: A composite score with routinely available parameters like age, TLC and CRP gives a good discrimination between infection and flare in a febrile patients. Development of low cost composite score if validated in the future in a larger cohort may improve our ability to differentiate infections and flare in patients with SLE.


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Etiology of elevated serum creatinine in patients with systemic lupus erythematosus at presentation and association of crescents in renal biopsy with rapidly progressive renal failure in a lupus cohort

Rajat Kharbanda, Able Lawrence, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, SGPGI, Lucknow, Uttar Pradesh, India

Background: Systemic lupus erythematosus (SLE) commonly involve kidney, with increase in creatinine due to lupus related or unrelated cause. Renal biopsy remains gold standard in diagnosis of lupus nephritis (LN). Higher number of crescents had significantly worse prognosis compared to no crescents.

Objectives: (1) Causes of impaired renal function in patients with SLE at presentation. (2) Association of crescents in renal biopsy with rapidly progressive renal function.

Methods: This was a retrospective single centre observational study where medical records of all SLE patients visiting tertiary care hospital 2001 to 2021 was reviewed. Data was retrieved from the case sheets, clinic files and electronic records using the hospital information system. The cause of raised serum creatinine (serum creatinine >1.5) was assessed. Renal biopsy of patients with impaired renal function was compared with renal biopsy of LN patients presenting with normal renal function. Data was analyzed using Strata 15, p value of <0.5 was taken as statistically significant.

Results: 1606 SLE clinic files were screened, of which 121 patients had elevated creatinine at presentation. Amongst the causes of elevated creatinine, lupus nephritis was most common (70.2%, 85/121) followed by infections (13. 2%, 16/121) [Table 1]. 68 renal biopsies of patient with elevated creatinine were compared with 319 renal biopsies with normal creatinine [Table 2]a. Proportion patients with presence of crescents was significantly higher in group with elevated serum creatinine compared to group with normal serum creatinine (51.4% vs 27.6%, p: 0.0001 and also, presence of crescents correlated with worsening renal function [OR: 2.7 (1.63-4.75)] [Table 2]b. Interstitial fibrosis and endocapillary proliferation were predictors of elevated serum creatinine in our cohort [Table 2]c.


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Conclusion: Apart from lupus nephritis as cause for increased creatinine, infections should always be screened. Crescents have significant association with elevated creatinine.


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Abnormalities in nail fold video capillaroscopy in pulmonary artery hypertension associated with lupus

Revanesh S Mirji, Ramya Janardana, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

Background: Nail fold video capillaroscopy patterns in patients with systemic lupus erythematosus (SLE) are not well described, especially those with concomitant PAH. It is unclear whether any particular NVC pattern or parameter predicts PAH development in SLE.

Objectives: (1) To evaluate NVC abnormalities in SLE-PAH patients. (2) To compare NVC findings in SLE-PAH and SLE non-PAH patients.

Methods: This is a single-center cross-sectional case-control study, wherein adult SLE patients with and without PAH (matched controls) were included. All patients were subjected to NVC using optilia digital capillaroscopy by the same evaluator taking standard precautions. The NVC findings of SLE-PAH were compared with that of SLE non-PAH using standard nomenclature and definitions. PAH was detected using transthoracic 2D echocardiography and defined as PASP >45 mmHg. Clinical Class I PAH patients were included.

Results: We included 16 SLE PAH and 16 SLE non PAH patients. Mean PASP was 58 mmHg versus 28 mmHg and mean TRV was 3.75 m/sec versus 2 m/sec respectively. The mean capillary density among SLE-PAH patients was 6.88 ± 1.82, the enlarged capillary was seen in 68.8%, the giant capillary in 18.8%, avascularity in 68.8%, microhemorrhages in 50%, tortuosity in 68.8%, neovascularization in 31.3%. The scleroderma pattern (SD) was seen in 25%, nonspecific pattern in 56.3 %, and the normal pattern is seen in around 18.8 %. There was no significant association of NVC findings or patterns with SLE-PAH patients or its severity [Table 1].
Table 1: Clinical and immunological profiling of systemic lupus erythematosus pulmonary artery hypertension and nonpulmonary artery hypertension patients and the nailfold video capillaroscopy parameters

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Conclusion: In our study we did not find any particular parameter of NVC to be associated with SLE PAH or its severity.


  POS071 Top


Severe thrombocytopenia in Indian inception cohort of systemic lupus erythematosus: Clinical association and outcome

Vineeta Shobha, Vasudha Bhat, Liza Rajasekhar1, Ashish J Mathew2, Chengappa Kavadichanda3, Manish Rathi4, Ranjan Gupta5, Amita Aggarwal6; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka, 1Department of Clinical Immunology and Rheumatology, Nizam Institute of Medical Sciences, Hyderabad, Telangana, 2Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, 3Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 4Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, 5Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, 6Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Prevalence of thrombocytopenia defined as platelet count (PC) less than <100,000/μL is reported in one-third to half of SLE patients and contributes to morbidity. higher damage and mortality.

Objectives: To study the frequency, associations, and outcomes in SLE patients with moderate-severe thrombocytopenia in our prospective cohort.

Methods: From the electronic database of nationwide, prospective, multicentre inception cohort (INSPIRE) of patients with SLE (diagnosed as per SLICC 2012 criteria), we analysed baseline clinical characteristics, laboratory parameter and disease activity in patients with moderate (PC 21-50,000/μL) and severe thrombocytopenia (<PC 20,000/μL). Patients with PC >150,000/μL at enrolment and who completed at least 6 months of follow up were categorised as controls. Cumulative dose of glucocorticoids (prednisone equivalent), immunosuppressive therapies employed, and variables associated with outcome at 1 and 6 months of follow-up were analysed.

Results: We included 2210 patients (F:M,11:1), median age being 26 years (IQR 20,34). About a quarter 542 (24.5%) patients had thrombocytopenia as per the SLICC2012 definition. However, at enrollment 230 (10.4%) patients had thrombocytopenia; moderate and severe thrombocytopenia was noted in 61 (26.5%) and 22 (9.5%) respectively. Overall, the bleeding manifestations were observed in 22/61 (36%) of moderate and 10/22 (45%) severe thrombocytopenia patients (P<0.001). Thrombocytopenia was significantly associated with autoimmune hemolytic anemia (P<0.001), leukopenia (P<0.001), lymphopenia (P<0.001) and low complement (P<0.05) as compared to control [Table 1]. Patients with moderate and severe thrombocytopenia were more likely to be treated with steroid pulse and had higher cumulative steroid use at 6 months [Table 2]. Mortality was higher in the severe thrombocytopenia category (p<0.05); however it was not an independent predictor of mortality. None of the patients died due to a major bleed.
Table 1: Distribution of clinical and laboratory parameters in systemic lupus erythematosus patients with moderate and severe thrombocytopenia, and controls

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Table 2: Treatment employed and outcome in systemic lupus erythematosus patients with moderate and severe thrombocytopenia and controls

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Table 1: Bivariate analysis showing correlation between thickened carotid intima media thickness with study variables

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Conclusion: Severe thrombocytopenia occurs in 12% of SLE patients and it is associated with shorter disease duration, other cytopenias and the requirement for high-dose glucocorticoid therapy.
Figure 1: Random forest feature importance model: Factors influencing carotid intima media thickness in study population

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  POS072 Top


Burden of atherosclerosis in Indian SLE patients: An observational study in a tertiary hospital of Eastern India

Rishav Mukherjee, Soumya Sarathi Mondal, Raja Bhattacharya; Department of General Medicine, Medical College, Kolkata, West Bengal, India

Background: SLE patients have an increased burden of atherosclerosis leading to adverse cardiovascular events. Indians are known to have greater predilection to develop insulin resistance and premature atherosclerosis. Carotid Artery Ultrasound has been recommended by the AHA/ACC to assess and follow progression of sub-clinical atherosclerosis by measuring carotid intima media thickness and correlate with traditional /non-traditional cardiovascular risk factors in SLE.

Objectives: To identify the at-risk population among SLE patients, with regard to atherosclerotic burden. To study the correlation between Carotid Intima Media Thickness, traditional/non-traditional CV risk factors in SLE.

Methods: A hospital based descriptive, cross sectional study was done in patients with SLE, diagnosed by the SLICC 2012 criteria, aged >12 years, irrespective of therapy status, were studied from April 2019 to August 2020, recruited by consecutive sampling. Non consenting patients, individuals with preexisting cardiovascular disease, past history of MACE, hypothyroidism (detected prior to diagnosis of SLE/disease onset), smokers, HIV patients, individuals with neck surgery and radiation, were excluded.

Results: 55 SLE patients were observed. Subgroup analysis was performed between SLE with Nephritis (36) and those without Nephritis (19) as presenting features. The mean age of the study subjects is 33 years with mean disease duration of 4.6 years. SLE nephritis patients had longer disease duration, younger age of disease onset, longer duration of steroid usage, higher mFRS scores and mean systolic BP. Framingham Risk scores were positively correlated with duration of disease, SLEDAI 2K scores and duration of steroid therapy [Figure 1]. The mean Carotid Intima media thickness of the study population is 0.91 mm with 10.9% plaque prevalence whereas mean CIMT of Lupus nephritis patients is 1.02+/- 0.27 mm but no statistically significant difference in CIMT was observed between the subgroups [Figure 1].
Table 1: The prevalence of depression and cognitive impairment in systemic lupus erythematosus

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Conclusion: In this study, patients with Lupus Nephritis/Neuropsychiatric SLE/ secondary APS, early age of lupus onset, longer disease duration with prolonged steroid therapy, significant proteinuria, higher anti-DSDNAAb levels and hypocomplementemia are observed to have higher mean CIMT and plaque formation.


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Prevalence of depression, and subclinical cognitive deficit in patient of SLE: A study from a tertiary health care centre

Smruti Ramteke, Sanjay Ramteke, Sandeep Yadav; Jasleen Hospital, Nagpur, India

Background: Significant number of systemic Lupus Erythematosus (SLE) patients have cognitive impairment and depression, which may or may not be part of Neuropsychiatric SLE. They may be clinical or subclinical, and their prevalence varies according to the tool used. Current literature on its prevalence lacks clarity, especially in the Indian community.

Objectives: This study aims to determine the prevalence of cognitive deficits in systemic lupus erythematosus (SLE) patients SLE.

Methods: It is a cross-sectional study. All SLE patients (SLICC criteria) attending the outpatient clinic were evaluated with Hamilton Rating Scale for Depression (HRSD) and Mini-mental state examination (MMSE).

Results: 250 patients of SLE attending an OPD clinic of a tertiary care hospital were evaluated. Based on Hamilton Rating Scale for Depression (HRSD), 89.6% of patients were depressed to some extent. Depression was mild in 43% of patients, moderate in 32.6 %, and severe in 14.8% [Table 1]. Based on mini-mental status examination scores, some cognitive impairment was found in 21.6 % of patients. Cognitive impairment was mild in 18.8% and moderate in 2.8 %, while none had severe.
Table 1: Hamilton rating scale

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Table 2: Hamilton rating scale

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Conclusion: Some amount of depression is found in almost every SLE patient, and about half have moderate to severe depression that may require treatment. One-fifth of SLE patients may have a cognitive impairment, and widespread use of diagnostic tools may help in early recognition.


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Correlation of cutaneous involvement with quality of life in systemic lupus erythematosus

Naga Hinduja, Sourav Padhee, Debasish Maikap, Prasanta Padhan, Ramnath Misra, C R Srinivas, Hemanta Kumar Kar, Sakir Ahmed; Department of Dermatology and Rheumatology, Kalinga Institute of Medical Sciences Bhubaneswar, Odisha, India

Background: Lupus erythematosus (LE) is an autoimmune disease with a variety of clinical manifestations ranging from multi-organ system involvement [systemic lupus erythematosus (SLE)] to limited cutaneous disease. We have assessed the impact on quality of life with skin involvement in SLE.

Objectives: To correlate the domains of SLEQoL with lupus disease activity score of skin involvement.

Methods: A cross-sectional study of 46 patients diagnosed with SLE was carried out to assess Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and quality of life score: SLE-QoL. Lupus disease activity and damage were measured using Mex-SLEDAI (Mexican SLE Disease Activity Index) and SLICC (Systemic Lupus International Collaborating Clinics) Damage Index respectively.

Results: In the study of 46 SLE patients, the mean age of the patients was 22.75 years (IQR: 19-41) with 35 females and 11 males. The CLASI score was 9 (IQR: 1-10) with an activity score of 4 (IQR: 1-5) and damage score of 3 (IQR: 0-3). The disease duration was 2.7 years (IQR: 2-3). Patients with active disease were quantified with Mex-SLEDAI of 8 (6.5-11.5) and SLICC was 1 (0-1). The correlation between CLASI and SLEDAI was 0.25. The correlation of different domains of the SLE-QoL with CLASI, and Mex-SLEDAI is presented in [Table 1] and correlation of different domains of the EQ-5D with CLASI, and Mex-SLEDAI is presented in [Table 2].
Table 1: Baseline characteristics and comparison with other cohorts

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Conclusions: In our cohort of lupus patients, quality of life did not correlate with CLASI overall but several of the components of SLE-QoL are potentially influenced by skin involvement and only activity domain of EQ-5D was influenced. Features of damage are more correlated with quality of life rather than acute skin disease activity.


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Clinical profile of children with lupus nephritis – study from a single centre in south India

Sruthi Ravindran1, Debasis Patro1, Jyothi Raghuram2,3, Anand P Rao1,3; 1Pediatric Rheumatology Clinic, Manipal Hospital, 2Aster Whitefield Hospital, 3Pediatric Rheumatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Background: Pediatric Systemic Lupus Erythematosus (pSLE) is a chronic multiorgan autoimmune inflammatory disease. Data on pSLE is sparse where the prevalence of nephritis is high (50-75%). 10% of patients will develop the end-stage renal disease (ESRD).

Objectives: To analyze the clinical and laboratory profile of children with lupus nephritis (LN) in a cohort of SLE patients attending a pediatric rheumatology clinic in South India.

Methods: We retrospectively reviewed 134 patients with SLE over a period of 10 years (April 2011-March 2021) in the Pediatric Rheumatology Clinic in Manipal hospital, Bangalore, India. 60 patients were having features of lupus nephritis as defined by the American College of Rheumatology (ACR). Biopsy-proven nephritis is defined as per the International Society of Nephrology (ISN) and the Renal Pathological Society (RPS) revised in 2018.

Results: The findings are summarized in the [Table 1]. 60% had renal involvement at the onset, 88.3% within 6 months, and 100% of patients had presented within 2 years of onset of the disease. Cyclophosphamide (27.6%) or mycophenolate mofetil (27.6%) or both (36.2 %) or azathioprine (19 %) were used as immunosuppressive agents. Rituximab was used in six patients and tacrolimus in three who did not respond to first-line therapy.

Conclusion: The majority of the children who develop nephritis, do so in the initial 2 years of the disease onset. Aggressive immunosuppression with a multi-disciplinary approach in the initial days of treatment can prevent end-stage renal disease in the future. Assess to tertiary care, financial constraints, and lack of awareness of pSLE in a developing country are major setbacks in the management of lupus.


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A single centre retrospective analysis of systemic lupus erythematosus patients affected with incident tuberculosis

S Shaaron, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Patients with Systemic Lupus Erythematosus (SLE) have a higher risk of developing TB. It would be helpful to identify risk factors for incident TB in SLE patients, the spectrum of TB in these patients, response to anti TB treatment and the effect of TB and its treatment on SLE.

Objectives: To describe the clinical features of SLE patients who developed incident TB. To describe the spectrum of TB in these patients and response to anti TB treatment. We also intent to study the effect of TB and its treatment on SLE.

Methods: Data of patients with SLE who had proven incident TB of Inpatients from 2007 to 2022 and outpatients from 2017 to 2022 in our institution, was collected from the Electronic Medical Records at our centre retrospectively and analysed.

Results: 30 adult patients with SLE had incident TB, of which, 28 were women (93%). Based on SELENA-SLEDAI at diagnosis of TB, 8 had no flare of SLE, 11 had mild to moderate flare, and 11 had severe flare. The median duration of diagnosis of TB since diagnosis of SLE was 60 months (range 9 -168 months) in no flare, 36 months (range 2-276 months) in mild and one month (range 0-60 months) in severe flare groups The median duration of use of steroid was 60 months (Range 2-120 months) in no flare group, 36 months (Range 0-154 months) in mild, 3.5 months (Range 0-60 months) in severe flare group. The mean steroid dose at diagnosis of TB in no, mild, severe flare groups were 16.5 mg, 15.72 mg and 8.4 mg respectively. The percentage of patients with disseminated / miliary TB was 50%, 54% and 18% no flare, mild flare severe flare groups respectively. 12 (40%) patients had pulmonary TB, 9 (30%) had disseminated TB and 9 (30%) had extrapulmonary TB. Four (13%) patients had genitourinary TB. Three (10%) had CNS TB and two (7%) had joint TB. Two of the 30 patients died during the hospital stay.

Conclusion: There is a high incidence of Extrapulmonary TB in SLE, particularly Genitourinary and CNS. The duration of diagnosis of TB after the diagnosis of SLE is shorter if the patient presents with a severe flare of SLE.
Figure 1: The bar diagram depicts the number of instances of tuberculosis per year compared to other serious infections through the year 2000-2020. The dashed line represents the 10 year moving average of all serious infections in the cohort and shows a consistently increasing trend

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Figure 2: The Kaplan Meier survival curve depicts time to first serious infection

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  POS077 Top


Serious infections in SLE- Incidence, associated factors, impact and trends over two decades

Rudrarpan Chatterjee, Sarit Sekhar Pattanaik, Durga P Misra, Vikas Agarwal, Able Lawrence, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, SGPGIMS, Lucknow, UP

Introduction: Infections are a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). We assessed the incidence and risk factors for major infections in adult SLE.

Methods: We carried out a retrospective review of 1354 patients of SLE (ACR 1997 criteria) with age ≥18 years visiting the rheumatology clinic at a single centre between 2000 to 2020. Clinical details were retrieved from clinical case records and supplemented with data from the hospital electronic health records. Serious infection was defined as need for hospitalisation, or infection resulting in disability or death. Cox regression was used to determine factors associated with serious infection and the effect of each serious infection on overall survival.

Results: Among the 1354 patients (1258 females) with a mean age of 30.32 ±9.42 years and a cumulative follow up of 7127.89 person years. A total of 439 major infections occurred in 339 (25.03% of the cohort) individuals at a rate of 61.6 serious infections per 1000 person-years of follow up. Recurrent infections occurred in 101 (7.45%) patients. The average number of infections increased from 9.9 to 23.7 per year from 2000-09 to 2010-19 [Figure 1]. Among the microbiologically confirmed cases (79.9% of cases) Tuberculosis (81) was the most common organism followed by S. aureus (65), E. coli (51), K. pneumoniae (29), P. aeruginosa (29), S. pneumoniae (20), Acinetobacter spp. (9), E. fecalis (9), Nocardia (4), Salmonella (3), Proteus (1) and Listeria (1). Forty (9.1%) opportunistic infections occurred: Cytomegalovirus (11), disseminated Herpes zoster (10), invasive candidiasis (5), cryptococcal meningitis (5), histoplasmosis (2) and 1 each of Aspergillus flavus pneumonia, CNS toxoplasmosis and Giardia lamblia. On multivariate analysis SLEDAI-2K (HR 1.02, 1.01-1.05), gastrointestinal involvement (HR 2.75, 1.65-4.69), current steroid dose (HR 1.65,1.55-1.76), average cumulative steroid dose per year (HR 1.007, 1.005-1.009) were associated with serious infection and higher albumin (HR 0.65, 0.56-0.76) was protective. Serious infection led to more damage accrual (1 vs. 0). Serious infection free survival at 1 year and 5 years was 82.9% (80.8-85) and 73.8% (71.1-76.6). There were 119 deaths with infection attributable mortality in 65 (54.6%). Hazard ratio for mortality was 18.2, 32.7 and 81.6 for the first, second and third serious infection [Figure 2].

Conclusion: Serious infections remain a major cause of mortality and damage accrual in SLE and are associated with higher disease activity, gastrointestinal involvement, hypoalbuminemia, current and cumulative steroid dose. TB prophylaxis in patients with SLE should be considered in endemic area especially when using high dose steroid therapy.


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Clinical profile and outcome of posterior reversible encephalopathy syndrome in systemic lupus erythematosus: A case series

Kaustav Bhowmick, Rashmi Roongta, Sumantro Mondal, Alakendu Ghosh, Parasar Ghosh; Department of Clinical Immunology and Rheumatology, Institute of Post-Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a neurologic emergency. Half of the patients have an associated autoimmune disease. It occurs rarely (<1%) in Systemic Lupus Erythematosus (SLE).[2]

Discussion: This study aimed to characterise the clinical, laboratory and neurological features of SLE patients with PRES. A retrospective analysis of SLE patients with PRES admitted between January 1, 2017 to June 31, 2022 was conducted. Fifteen such patients were identified. All were females. Age of onset of PRES ranged from 13 to 64 years (median 30.5 years). The median duration of SLE before the onset of PRES was 66 months (range 1 to 108 months). The commonest symptoms were seizures and altered sensorium (n=13 each). Two patients presented with headache, vomiting and reduced vision. The commonest sites involved on MRI were the occipital lobes (n=15) and parietal lobes (n=12). Atypical sites involved were basal ganglia, lateral ventricle, thalamus, pons and corpus callosum. Nephritis was present in 14 patients (93.3%) with one-third having nephrotic range proteinuria. The hematological domain was involved in two-thirds of the patients with lymphopenia in one-third and Coomb's positivity in half. The mean SELENA-SLEDAI - N score (disease activity minus activity in neurologic domain) was 19.8 ± 9.9. Thirteen (86.7%) were hypertensive and required a mean of 2.9 antihypertensive agents per day. Two patients were normotensive. All patients were serologically active with low C3 and high dsDNA titres. All had complete clinical recovery except one who had persistent ataxia. Twelve (92.3%) of the thirteen patients presenting with seizures received antiepileptic drugs for a mean duration of 9.3 ± 5.5 months.
Figure 1: Overview of cohort demonstrating SLE subsets

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Conclusion: PRES was associated with high extra-neurological disease activity in the renal and hematological domains. Seizures were the commonest presenting manifestation and the parieto-occipital areas were most commonly involved. Majority were hypertensive at onset and required multiple antihypertensives.


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Antiphospholipid antibodies and pulmonary hypertension in systemic lupus erythematosus

Sandra Manuel, V Nayana, Sasikala Bheemireddy, Ramya Janardana, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

Background: Pulmonary hypertension (PH) is well recognised as non-thrombotic association of aPL. In SLE, reported prevalence of PH is 12.3% versus 7.3% in aPL-positive vs. aPL-negative SLE respectively, the overall odds ratio being 2.28, highest risk for lupus anticoagulants and IgG anticardiolipin antibodies.

Objectives: We aim to study the prevalence of aPL in newly diagnosed SLE patients with PH as well as to identify any specific aPL group association, if any.

Methods: We included all consecutive newly diagnosed SLE patients at SJMCH, Bangalore between 2018-22 with complete aPL analysis and echocardiogram available. aPL was detected using standard methods (anticardiolipin and antiB2GP1 antibody by ELISA and Lupus anticoagulant as per the ISTH-SSC 2009 guideline). PH was detected using transthoracic 2D echocardiography and defined as PASP >39 mmHg. Patients were subgrouped based on presence or absence of PH and aPL.

Results: We evaluated 229 SLE patients and identified 29 (12.6%) patients with PH [Figure 1]. Among patients with PH, mean PASP was 55.6 ± 18.3mmHg. Of them 10 (34.5%) patients had at least one subtype of aPL positivity, while the rest (n=19, 8.2%) tested negative. Amongst aPL positive patients (82/229), 72 (31.4%) patients did not have PH. Isolated B2GP1 positivity was encountered in 4/10 patients (p=0.8) and Isolated LA positivity in 5/10 patients (p=0.114). No association was noted with Isolated ACL (p=0.503). Only single PH positive patient had dual aPL positivity while none from the triple aPL positive group had PH. There was no difference in clinical characteristics, autoantibody prevalence, disease activity and mortality between these groups [Table 1].
Table 1: Prevalence of pulmonary hypertension and its association with antiphospholipid antibodies positivity

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Conclusion: Most SLE cohort studies have described PH as a non-thrombotic association of aPL, however, we were not able to find any association of PH to aPL positivity.


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A study of hematological involvement in SLE in patients in a tertiary care centre in central India

Upasana Agarwa, S S Nelson; Department of Medicine, NSCB Medical College and Hospital, Jabalpur, Madhya Pradesh, India

Background: Hematological profile of SLE patients is understudied and given less importance compared to its prevalence in patients of SLE. Studies conducted in various regions show significantly higher involvement of blood related disorders than expected.

Objectives: To see the hematological involvement in SLE patients in a tertiary care centre in Central India.

Methods: 50 patients of SLE fulfilling the ACR and EULAR criteria (2018) from 1st January2021 to 30th June 2022.

Study Design: Single Center Prospective Observational Correlational Study.

Results: The female:male ratio in our study was 24:1. The most common symptoms were malar rash, oral ulcers and alopecia. More than 80 % patients were found to have anemia on routine workup. The most common anemia found was Iron deficiency Anemia. Leucopenia presented most commonly with lymphocytopenia. Few patients presented with thrombocytopenia. Lupus nephritis was found in more than 30% patients [Table 1].

Conclusions: Hematological abnormality is the most common finding in patients of SLE in Central India. Refractory cytopenias directly correlate with disease activity and necessitate immunosuppressive drugs.


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Short-term outcome in patient of lupus nephritis treated with multitarget therapy (MMF +Tacrolimus): An experience from a tertiary health care centre in Mumbai

S Yadav, R Samant, A Patankar1, G Kakade2, S Singh3, A Zanwar4; Department of Rheumatology, P. D. Hinduja National Hospital and Medical Research Centre, 3Department of Rheumatology, Kokilaben Dhirubhai Hospital, Mumbai, 1Department of Rheumatology, Jupiter Hospital, 2Department of Rheumatology, Sahyadri Super Speciality Hospital, 4Poon Super Speciality Clinic, Pune, Maharashtra, India

Introduction: The complete remission rate for lupus nephritis (LN) with current induction treatment regimens remains inadequate (5.8–56.2%), with a significant number of patients progressing to ESRD. Data on multitargeted therapy (MTT) (MMF+Tacrolimus) are primarily from the Chinese population, while data from the Indian subcontinent are scarce.

Aim: Short-term outcome in patient of lupus nephritis treated with multitarget therapy (MMF +Tacrolimus).

Methods: Biopsy-proven lupus nephritis patients treated with MTT were retrospectively analysed for clinical profile, indication for treatment, and response to therapy according to KDIGO outcome criteria.

Results: 11 (10 females) patients with biopsy-proven lupus nephritis were included for analysis. Mean age of onset of lupus nephritis was 24.91 ± 8.1 years. Renal biopsy showed class 4 LN in nine patients and class 5 in two. Mean baseline urine protein creatine ratio (UPCR) was 6.8 ± 3.3. Mean baseline serum creatinine was 0.7 ± 0.09 mg%. Indication for using the MTT regime was refractory to the initial induction regime of MMF 2-3 gm /day for at least six months in nine (9/11) patients and relapse on MMF 2-3 gm maintenance therapy in two (2/11) patients. The mean follow-up duration was 8.7 ± 8.1 months. At 3-months, 1/11 (9%) had Complete response (CR), 8/11 (73%) had response (PR), and 1/11 (9%) failed to respond. At six months, 1/6 (17%) had CR, 4/6 (66%) had PR, and 1/6 (17%) failed to respond. At 12 months, 2/4 (50%) had CR, and 2/50 (50%) had PR. At 1- year, the drug was discontinued for gastrointestinal disturbance with tremors in one patient and pulmonary nocardiosis in one. In three patients, initial response to MTT was followed by relapse at variable intervals even on treatment [Table 1].
Table 1: Clinical profile and treatment outcome of patient treated with multitargeted therapy

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Conclusion: Multitargeted therapy might be effective in refractory cases of lupus nephritis who have failed initial induction therapy. They may also be effective in the patient who relapsed on immunosuppression. Long-term data and head-to-head trials are needed to evaluate its efficacy in the Indian population.


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Occurrence and causes of hospitalization in patients of SLE in a tertiary care hospital

Jyotsna Oak, Arjun Das; Department of Rheumatology, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disorder affecting many organs in which the tissues and cells are damaged due to pathogenic autoantibodies and immune complexes. The flares of the disease are characterised by severe organ involvement and is a major factor requiring admission in the hospital for SLE patients and it ranges from 11-80.8%.[2] About 10.9 to 37% of hospitalisations are due to infection.[3] We conducted the study to analyse the pattern of admissions in SLE patients to help determine the economic burden of SLE admissions.

Objectives: To determine factors affecting morbidity and mortality in a contemporary cohort of hospitalised patients and estimate the rate of SLE hospitalisation.

Methods: A retrospective chart review was undertaken for all the patients admitted in tertiary care center between April 2012 to December 2021.

Results: There are a total of 122 patients of SLE meeting inclusion criteria. Out of these, 41 needed hospitalization during this period. Average age at diagnosis was 38.24+/-12.63 years (SD) at hospitalisation. 95.08% were females and the remaining we 4.92% were males. The most common reasons for hospitalization included SLE Flare 18.85% (23/122) and infection 16.39% (20/122). Acute coronary syndrome 3.27% (4/122) and pulmonary and venous TE 1.63% (2/122) were less common in our patients. ICU admission was needed in 60.97% (25/41). The factors responsible for hospitalization were analysed and include: cardiac pathology, respiratory pathology, thrombocytopenia, haematological, neuropsychiatric and musculoskeletal involvement. [Table 1] also shows their percentage of occurrence and p value.
Table 1: Factors responsible for hospitalization in systemic lupus erythematosus patients

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Conclusion: In this contemporary cohort, SLE flare and infection remain the prime reasons of hospitalization. Causes of admission and length of stay are consistent with previous studies.


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Pattern of NFC changes seen in lupus and its correlation with organ involvement and complication

Abhilasha Manwatkar, Ruchika Goel, B Antonisamy, Shivraj Padiyar, S Chandu, John Mathew; Department of Clinical Immunology and Rheumatology and Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India

Background: Systemic lupus Erythematosus (SLE) is an autoimmune disease characterised by vascular complications. Nail fold capillaroscopy (NFC) is a simple, easy and non-invasive method. NFC detect real-time microvascular changes.

Objective: To detect NFC abnormalities (by qualitative and semi quantitate methods) in SLE and its correlation with clinical and laboratory parameters.

Method: This was a descriptive study of SLE patients from rheumatology clinic in a tertiary care hospital in India. Patients' demographics, clinical data, disease activity and serology were recorded. Disease activity was assessed by SELENA-SLEDAI. NFC changes in SLE patients were recorded by both qualitative and semiquantitative methods (NFC score). NFC score used by Riccieri et al. 2005.

Results: Seventy-eight patients were included; the median age of 31 years (IQR 25-39), and 70 patients (90%) were female. At the time of NFC thirty-three (42%) patients had disease activity. Forty-five (57%) showed NFC abnormality and 33 did not. The distribution of NFC changes is shown in [Figure 1] Decreased capillary density is seen in 45 (58%) of patients. Avascular area was seen in twenty-two (28%). The most common morphological abnormality was enlarged capillaries in eight (10%). Arthritis was associated with higher NFC score (P=0.025). Pulmonary artery hypertension (PAH) (P=0.004), digital ischemia (P=0.01) and arthritis (P=0.02) are associated with decreased capillary density. Out of various parameters, micro bleeding was found to be associated with PAH (P=0.01) and Antiphospholipid serology (Beta 2 glycoprotein IgM, IgG, P=0.02). There was no association between disease activity and NFC changes.
Figure 1: Nail fold capillaroscopy features seen in systemic lupus erythematosus

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Conclusion: Majority of lupus patients had NFC abnormality. NFC can be used as an important tool in personalised medicine to identify SLE patients with high risk of organ involvement and complications especially PAH, arthritis and cardiac involvement.


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A study of the clinical profile of systemic lupus erythematosus patients with special reference to pulmonary manifestations

Melvin C Anto, Bhaskar Thakuria; Department of General Medicine, Gauhati Medical College, Assam, India

Background: SLE is an autoimmune disease in which organs, tissues and cells undergo damage mediated by tissue binding auto antibodies and immune complexes. Many of its clinical manifestations are secondary to the trapping of antigen antibody complexes in capillaries of visceral structures or to auto antibody mediated destruction of host cells. Pulmonary involvement is common in SLE and will affect half of patients during disease course and is part of the spectrum of presenting symptoms in 4% to 5% of patients. Subclinical involvement is also prevalent evident by abnormal pulmonary function tests in patients who are free from respiratory symptoms, other complications of lupus, like cardiac failure or nephritic syndrome may lead to lung involvement with pleural effusion.

Objectives: To study the pulmonary manifestations in systemic lupus erythematosus patients and to correlate the clinical features with plain radiograph, pulmonary function tests (PFTs) and high resolution computed tomography (HRCT), and to correlate the SLE lung manifestations with lupus nephritis.

Methods: A hospital based observational analytical cross sectional study was done with data collected over 1 year. The study includes patients over 12 years diagnosed with SLE on the basis of EULAR- ACR 2019 criteria.

Results: Out of 75 patients with SLE, female patients were more affected and have symptoms like pleuritic chest pain being more common, followed by dyspnoea, with pleural effusion having slightly incidence than ILD. The detection of pulmonary manifestations by HRCT thorax is more sensitive than chest X ray and PFTs. The patients with lupus nephritis had slightly increased incidence of pulmonary manifestations than without lupus nephritis.

Conclusion: Pulmonary involvement is present in significant number of SLE patients and were symptomatic. Common manifestations were pleural effusion and ILD; pleural effusion being slightly more common.


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Gastrointestinal manifestations in systemic lupus erythematosus are associated with high disease activity and mortality: A nationwide cohort study from India

Pankti Mehta, Akansha Khare1, Amita Aggarwal1, Liza Rajasekhar2, Chengappa Kavadichanda3, Vineeta Shobha4, Ashish J Mathew5, Parasar Ghosh6, Bidyut Das7, Avinash Jain8, Ranjan Gupta9, Manish Rathi10 for the INSPIRE Group; Department of Clinical Immunology and Rheumatology, King George's Medical University, 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 2Department of Clinical Immunology and Rheumatology, Nizam Institute of Medical Sciences, Hyderabad, Telangana, 3Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 4Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, Karnataka, 5Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, 6Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, 7Department of Rheumatology, SCB Medical College, Cuttack, Odisha, 8Department of Clinical Immunology and Rheumatology, SMS Medical College and Hospital, Jaipur, Rajasthan, 9Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, 10Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Gastrointestinal (GI) manifestations of Systemic Lupus Erythematosus (SLE) although rare are often severe. These are difficult to directly attribute to SLE as they can also be associated with drugs, infections and other organ manifestations of SLE. There is a paucity of prospective data on the same and thus, we studied the GI manifestations in a prospectively enrolled nationwide cohort of SLE in India.

Methods: Cases with GI manifestations (defined as per BILAG) along with controls were selected from the database of a nationwide, prospective, multicenter cohort (INSPIRE) of patients with SLE. Data on demographics, clinical features, investigations, disease activity at baseline and early outcomes were retrieved for analysis. Data are expressed as mean ± standard deviation and SPSS was used for statistical analysis.

Results: Of 2210 patients with SLE enrolled, 254 (11.5%) had GI manifestations, with more than one GI feature in 39 patients. The most common was ascites (193,76%) followed by lupus enteritis (35, 13.8%), lupus pancreatitis (32, 12.6), lupus hepatitis (19, 7.5), lupus peritonitis (6, 2.3), intestinal obstruction and lupus cholecystitis (3, 1.2%) with malabsorption and protein losing enteropathy being the least common (2, 0.7%). The majority were observed within 2.2 ± 3.3 months of presentation with 97 (38%) being present at the onset. On univariate analysis, patients with GI manifestations were significantly younger; with higher frequency of fever, lupus nephritis, psychosis, seizures, pleural effusion, anemia, lymphopenia, thrombocytopenia, elevated creatinine, hypoalbuminemia and hypocomplementemia as compared to controls. There was no difference in the serology between the two groups. SLEDAI 2K and the BILAG were higher in the cases [Table 1]. On multivariate analysis, younger age [p=0.03, 0.97 (0.95-0.99)]; high creatinine [0.001, 1.6 (1.2-2.1)], hypoalbuminemia [0.0001, 0.6 (0.5-0.8)], pleural effusion [0.0001, 3.3 (1.9-5.2)], thrombocytopenia [0.02, 1.6 (1.1-2.5)] were significantly associated with GI features [Figure 1]. Mortality was higher in the cases [10.3 and 5.5%, p=0.04, 1.9 (1.1-3.7)] as compared to controls [Table 1].
Figure 1: Association of gastrointestinal manifestations on multivariate analysis as compared with controls

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Table 1: Comparative analysis between systemic lupus erythematosus patients with and without gastrointestinal features

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Conclusion: GI manifestations were observed in 11.5% of the cohort with ascites being the most common feature. GI involvement is associated with higher disease activity and mortality.


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Antibodies to dsDNA and associated proteins: Association with proteinuria and lupus nephritis

Ranjan Gupta, Amita Aggarwal1, Avinash Jain2, Liza Rajasekhar3, Chengappa Kavadichanda4, Vineeta Shobha5, Ashish J Mathew6, Parasar Ghosh7, Bidyut Das8, Manish Rathi9 for INSPIRE Group; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 2Department of Clinical Immunology and Rheumatology, Sawai Mansingh Medical College and Hospital, Jaipur, Rajasthan, 3Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, 4Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 5Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, Karnataka, 6Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, 7Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, 8Department of Rheumatology, SCB Medical College, Cuttack, Odisha, 9Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Anti-dsDNA antibodies (ADA) are traditionally measured by ELISA and are associated with Lupus nephritis (LN). Testing by immunoline assay (IL) provides additional information on antibodies to dsDNA, nucleosomes and histones also.

Objectives: (1) Does ADA by IL show similar association with proteinuria (i.e. lupus nephritis; LN) and proliferative LN (PRLN) as by ELISA? (2) Does co-occurrence of these 3 antibodies have any impact on association of ADA with LN or PRLN?

Methods: Autoantibody profiles on IL and ADA values by ELISA (Euroimmune, Germany) of 1907 Indian SLE patients (F:M=1749:158; mean age=27.5 years) at baseline were used. Among patients with LN (n=734), renal biopsy was available for 412 patients (251 PRLN and 161 non-proliferative LN). Chi-square test was used to find association of individual and combination of antibodies with LN and the 2 biopsy classes.

Results: ADA on ELISA and IL tested positive in 557 (74.96%) and 296 (39.83%) patients of LN respectively. ADA results, as such, by both methods showed significant association with LN and/or PRLN but this association disappeared when re-tested after omitting the co-positivity for the other two antibodies. Isolated positivity for the other 2 antibodies also didn't show any association. Double positivity for ADA (IL) and anti-nucleosome antibodies showed a significant association with PRLN. Triple antibody positivity in both the groups (based on ADA testing methods - ELISA and IL respectively), showed significant association with LN and PRLN [Table 1]. Positive predictive value of ADA (ELISA and IL respectively) for LN versus PRLN (41.96% and 11.96% vs. 34.04% and 12.06%) was lesser than that of triple positivity in either group (43.13%% and 18.18% vs. 42.92% and 20.38%).
Table 1: Association of antibodies to dsDNA, nucleosomes and histones (alone or in combination) with proteinuria and proliferative lupus nephritis

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Conclusions: ADA positivity alone (ELISA or IL) doesn't have any association with LN or PRLN and the odds of developing renal involvement increase significantly with the co-positivity of antibodies to nucleosomes and histones with ADA.


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Fc gamma receptor polymorphism in SLE: Association with clinical phenotypes and disease severity

Sonalika Sahoo, Sukanya Priyadarshini Mohanty, Sarit Sekhar Pattanaik1, Saumya Ranjan Tripathy1, Rina Tripathy2, Bidyut Kumar Das1; Departments of General Medicine and 1Clinical Immunology and Rheumatology, SCB MCH, 2Department of Biochemistry, SCB MCH and SVPGIP, Cuttack, Odisha, India

Background: Multiple genes with alleles increasing risk for SLE have been identified, of which FCYRs mutation has been of growing interest. Over the past few years, correlation of Fc Gamma receptor allelic polymorphisms with inherited differences in susceptibility to SLE and its diverse clinical manifestations have been extensively studied.

Objectives: This study aims to analyse polymorphism of Immunoglobulin gamma Fc region inhibitory receptor IIB in predisposition to SLE and its corelation with different clinical phenotypes and disease severity. The study also aims to explore the possible role of FCYRIIB mutation in protection against malaria, which in turn has led to enhanced susceptibility to SLE.

Methods: A total number of 90 patients fulfilling the SLICC criteria along with 65 age and sex matched healthy controls were recruited for the study. We studied the clinicodemographic pattern of these patients and the association of FCYRIIB232T polymorphism with SLE.

Results: Although, the prevalence of FCYRIIB-T232T genotype (Homozygous) was higher in lupus patients compared to healthy controls, the results didn't reach statistical significance. The comparison between various clinical phenotypes and genotypes didn't reveal difference between the groups. There was no correlation between genotype and various measures of disease activity like C3, C4 and SLEDAI. Interestingly, patients with a homozygous mutation had significantly higher levels of CRP compared to the other two subgroups.

Conclusion: The current study showed higher prevalence of FCYRIIB-T232T genotype (Homozygous) in lupus patients compared to healthy controls, but the results were statistical insignificant. The comparison between various clinical phenotypes and genotypes didn't reveal difference between the groups. There was no correlation between genotype and the various measures of disease activity like C3, C4 and SLEDAI. This finding needs to be validated in a larger cohort of patients.


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Gray matter myelitis is a severe phenotype of myelitis in systemic lupus erythematosus: Experience from a tertiary care hospital in South India over a decade

Ritasman Baisya, G S R Murthi1, Phani Kumar, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, 1Indian Institute of Technology, Hyderabad, Telangana, India

Background: Myelitis is a severe yet rare manifestation of SLE. Literature on lupus myelitis is limited to case reports and series. Birnbaum et al. reported the largest series (n=22) with two distinct phenotypes based on clinical and imaging findings with mechanistic and therapeutic implications.

Objectives: To identify distinct phenotypes in lupus myelitis and compare activity and outcomes with the formulation of a prediction model for survival.

Methods: This is a retrospective study, data of 35 lupus myelitis with follow-up were extracted from the lupus registry (N=1550) in the last 15 years (2007-2022). Gray matter myelitis (GMM) (i.e., flaccidity, hyporeflexia) and white matter myelitis (WMM) (i.e., Spasticity, hyperreflexia) were two subtypes. Disease activity was assessed by SLEDAI-2K and outcome by death/recurrence/disability - Modified Rankin score (MRS). MRS at discharge and SLEDAI at admission were used as predictors for survival outcomes among subtypes.

Results: Median age at presentation was 24.5 years (12.5-36.5), female-male ratio 32:3, 24 patients presented with GMM while eleven with WMM. GMM had more CNS events (p-0.02), high SLEDAI (p-0.021) and extra-CNS involvement (p>0.05). Anticardiolipin antibodies were more in WMM (p-0.03) [Table 1]. Weibull survival probability plot showed that 25%, 50 % and 75% of GMM patients had the probability of death within 13, 85, and 376 months from the myelitis episode while the same for WMM is 96, 256, 556 months respectively, implying GMM has a worse survival outcome [Figure 1]. Using linear regression, MRS at discharge and SLEDAI can predict survival status in GMM (R=0.64, p= 0.04), not in WMM (R= 0.42, p= 0.46).
Figure 1: Weibull probability plot for life status and Kaplan Meyer curve of survival GMM versus WMM - 25%, 50 % and 75% of patients with GMM had a chance to die within 13, 85, 376 months from the first attack while the same for WMM is 96,256, 556 months respectively implying grey matter myelitis has a worse survival outcome. However, Kaplan Meier's plot showed a mean survival time more for GMM but not statistically significant. (probably due to the small sample size)

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Table 1: The difference between gray matter myelitis versus white matter myelitis

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Conclusion: Lupus myelitis has two distinct subtypes, GMM being associated with more active disease, poor survival, and significant disability, and its survival status can be predicted by MRS and SLEDAI scores.


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Clinical and autoimmune profile of systemic lupus erythematosus patients in a tertiary care centre in Central India

Silas Supragya Nelson, Pawan Soni, Raghuram Mahishi; Department of Medicine, Netaji Subhash Chandra Bose Medical College and Hospital, Jabalpur, Madhya Pradesh, India

Objective: The objective of this study is to see the clinical profiles and frequency of autoantibodies in Systemic Lupus Erythematosus (SLE) patients in a tertiary care centre in Central India and also the disease activity (SLEDAI) in SLE patients in a tertiary care centre in Central India.

Materials and Methods: Thirty seven patients of SLE were considered for the study. Antinuclear Antibody (ANA) profiles and titres of autoantibodies were determined using the ANA blot, immunofluorescence assays and enzyme linked immunosorbent assays. Also the clinical presentations were documented in all patients.

Results: Haematological (83%), muco-cutaneous (67.5%), constituitional (59.5%), renal (43.2%) and musculoskeletal (35.1%) were the common clinical presentations, while among autoantibodies, Antinuclear antibodies (ANA) were present in 100% of patients, anti dsDNA antibodies in 91.6% of patients, anti Sm in 32.4%, low C3 in 24.3% and low C4 in 18.9% of patients along with other autoantibodies in varying proportions. The average age affected was 28.9 years with high female preponderance (F:M=17.5:1). The average SLEDAI was 15.7 [Figure 1] and [Table 1].
Figure 1: Neuroimaging of the patient. (a) T1 postcontrast sagittal MRI showing diffuse smooth dural enhancement (white arrow), engorgement of the sagittal sinuses (orange arrow). (b) T1 postcontrast axial MRI showing rounding of the dural sinus consistent with engorgement of the sagittal sinus (blue arrow). (c) Vertebral bony spur (yellow arrow). (d) CT myelography showing D2/D3 small dural leak

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Table 1: The clinical characteristics of primary central nervous system vasculitis patients (n=26)

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Conclusion: The most common clinical presentations in the study were haematological, muco-cutaneous, constitutional (fever), renal and musculoskeletal, while ANA was the most coomon autoantibody found in SLE patients, followed by anti dsDNA antibody.


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Spontaneous intracranial hypotension: A rare cause of headache in a patient of systemic lupus erythematosus

Upendra Rathore, Vivek Singh, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Spontaneous Intracranial Hypotension (SIH) is defined as opening CSF pressure of less than 7 mm H2O (Normal 7 to 20 mm H2O). It classically presents with headache in uprights position and improves on lying down position. SIH is most commonly caused by the spontaneous dehiscence of a meningeal diverticulum or as a consequence of dural tears secondary to degenerative changes of the vertebral column. Identifying a CSF leak can be challenging, especially in the setting of SIH. The leak can occur anywhere along the neuroaxis. Furthermore, the leak can be slow, intermediate, or high-flow.[1] There are few case reports showing spontaneous CSF leak in patients with SLE.

Discussion: We present a case of a 27 year old female, k/C/O SLE with lupus nephritis currently in remission presenting with complaints of postural headache which improved on lying down. There was no history of blurring of vision, diplopia, floaters, flashes, watery discharge from ear or nose, fever, altered sensorium, irrelevant talking, seizures or any features to suggest lúpus disease activity. Her C3,C4 and dsDNA were normal, opening CSF pressure was 6 mm HHg with 5 cells (all lymphocytes). Currently she was on HCQs and tacrolimus. MRI Brain [Figure 1]a and [Figure 1]b was suggestive of intracranial hypotension with diffuse pachy-meningeal enhancement with engorgement of dural venous sinuse. An incidental finding of vertebral bony spur [Figure 1]c was also seen which would have contributed to traumatic CSF leak. CT myelography [Figure 1]d demonstrated a leak a D2-D3 level. The patient underwent autologous blood patch to seal the CSF leak. Postoperatively her symptoms improved and she is doing fine on 6 months of follow up.
Figure 1: (a) Long segment narrowing of middle cerebral artery of a patient. (b) The narrowing almost completely improved after treatment with steroid and mycophenolate for 14 months

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Conclusion: Headache should not be taken lightly in any patient of SLE, detailed history, examination and prompt investigations helps to clinch the diagnosis.


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Efficacy of mycophenolate in primary central nervous system vasculitis of adults: An observational study

Shyamashis Das, Debanjali Sinha, Rudra Prosad Goswami1, A Shobhana2, Sukalyan Purkayastha3; Departments of Rheumatology, 2Neurology and 3Neuro-Intervention, Institute of Neurosciences, Kolkata, West Bengal, 1Department of Rheumatology, AIIMS, New Delhi, India

Background: Primary central nervous system vasculitis (PCNSV) is a rare disease characterized by inflammation of the blood vessels of brain and spinal cord. There is no consensus regarding the optimal immunosuppressive therapy in this disease.

Objective: To observe efficacy of mycophenolate as induction and maintenance immunosuppressive therapy in PCNSV.

Methods: Adult patients with PCNSV, diagnosed by Calabrese's criteria, were recruited prospectively from 2017 to 2021. All had neurodeficits and MRI/CT lesions in brain and typical abnormalities in digital subtraction angiography (DSA). All patients were treated with methyl-prednisolone pulse followed by oral prednisolone and mycophenolate and received standard care of stroke.

Results: A total of 26 cases were recruited as per inclusion and exclusion criteria. The median age at presentation was 39 years (IQR: 34-49) with a slight female predilection (61.5% females, 16/26). DSA abnormalities confined to small vessels were noted in 11.5%, in large vessels only in 42.3% and in both in 46.2%. All scans showed multifocal abnormalities. Arteries involved in order of frequency were MCA (84.6%), ACA (76.9%), ICA (42.3%), PCA (38.5%) and the VA (7.7%). DSA was repeated in 16 patients after a median duration of 13 months (10.5-19.7), out of which 10 (62.5%) showed improvement [Figure 1]. Median time to improvement in DSA was 19 months (95% CI: 9-29). Brain biopsy was done in five patients, all of whom had typical DSA features: granulomatous inflammation without amyloid deposit was seen in three samples and perivascular lymphycytic infiltration in one. Median duration of follow-up was 24.5 months (14.25-38). The Modified Rankin Score (mRS) of 0 was achieved in 38.5% (10/26) and mRS of 1 was achieved in 69.2% (18/26) patients. The median time to achieve a mRS of 1 was 12 months (95% CI: 6.8-17.2). Three patients experienced a relapse after initial response, two after six months and one after two years [Table 1].
Table 1: Comparison of clinical profile of patients with early onset disease (<5 years) and patients with disease onset after 5 years

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Conclusion: Mycophenolate resulted in clinical improvement as well as angiographic improvement or non-progression in PCNSV.


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Childhood onset polyarteritis nodosa: A clinical experience over 32 years from a tertiary care referral center in North India

Archan Sil1, Ankur Kumar Jindal2, Suprit Basu1, Deepti Suri3, Anju Gupta3, Amit Rawat3, Manphool Singhal4, Rajesh Vijayvergiya5, Pandiarajan Vignesh6, Michael Steven Hershfield7, Surjit Singh8; Department of Pediatric Allergy and Immunology, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Department of Cardiology, Advanced Cardiac Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India, Department of Medicine, Duke University School of Medicine, Durham, North Carolina

Background: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized vessels. Although there are few anecdotal reports of childhood-onset PAN (c-PAN) from India, such a large cohort with long duration of follow up has never been reported.[2]

Objectives: To report and analyze the demography, clinical characteristics, laboratory features, treatment plans and outcomes in a c-PAN cohort from North India.

Methods: Data were collated from clinic files of patients registered at the Pediatric Rheumatology clinic from the year 1990 to 2022. Statistical analysis was done using SPSS software.

Results: In a cohort of 40 patients, male to female ratio was 3.4:1, with median age of onset of 7.45 years (Range: 1 – 13.7 years). Hepatitis-B was found to be associated in 2 cases. Deficiency of adenosine deaminase 2 (DADA-2) was the final diagnosis in 6 (15%) patients. The most common manifestation was fever (82.5%) and hypertension (82.5%). Neurologic involvement was seen more frequently (67.5%) than cutaneous manifestations (62.5%). Raised ASO titres were documented in 7 patients (17.5%). Computed tomography (CT) angiography done in all patients revealed renal artery to be the most common vessel involved (45%). Methylprednisolone pulse was required in 38 patients (95%), with additional requirement of cyclophosphamide in 16 patients (40%). Azathioprine and mycophenolate mofetil were used as maintenance therapy in 15 (37.5%) and 10 (25%) patients respectively. Two patients (5%) with refractory PAN received injection adalimumab. Number of relapses was significantly more in patients with younger age (onset of disease at or before 5 years of age) (p =0.003) than the rest of the cohort.

Conclusions: c-PAN is a rare entity. [3,4] This is the largest single-centre cohort of patients to be reported from India. Analysis of our cohort revealed that patients with early onset of disease (<5 years) had more number of relapses than their older counterpart.


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Clinical profile and follow-up of childhood onset polyartritis nodosa in a tertiary care centre in South India

Anil Kumar Tennelli1, Debasis Patro1, Jyothi Raghuram2,3, Anand Prahalad Rao1,2; 1Manipal Hospital, 2Indira Gandhi Institute of Child Health, 3Aster Hospital, Whitefield, Bengaluru, Karnataka, India

Introduction: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly affecting medium-sized vessels. Though it is a multisystem disease, it predominantly involves the skin, gastrointestinal tract (GIT), musculoskeletal system (MSK), and kidneys. Cutaneous PAN is a limited form of the disease wherein the disease remains limited to the skin without any visceral organ involvement.

Aims and Objectives: To study the clinical profile, laboratory findings, and outcome of childhood-onset PAN.

Methods: A retrospective study of 20 children fulfilling the European league against rheumatism (EULAR)/ Paediatric rheumatic European Society (PRES)/ Paediatric Rheumatology International Trials Organization (PRINTO) classification criteria of PAN who were seen over a period of 9 years.

Results: The findings are summarized and compared in the [Table 1]. The median age of onset of symptoms was 9.4 years (Range: 4- 15 years) with the M: F ratio being 1:1. The most common presentation is skin involvement (100%) followed by fever (95%) and MSK manifestations (80%). Deficiency of Adenosine Deaminase 2 (DADA 2) was detected in two cases. Labs revealed anemia in 50%, leucocytosis in 65%, and thrombocytosis in 30%. In follow-up, three patients were in complete remission whereas two were lost follow-up. 9 patients had relapsed on medication whereas 6 patients had relapsed off medication. The mean duration of treatment was 3.5 years. One child succumbed due to a complicated varicella infection and another patient died due to an unrelenting PAN.
Table 1: Baseline characteristics and comparison with other cohorts

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Conclusion: Childhood PAN is a chronic pediatric vasculitis characterized by dermatologic and musculoskeletal manifestations. Early diagnosis and treatment may minimize sequelae in patients with PAN. Relapses occurred more frequently in those with systemic involvement and poor compliance with medication.


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Clinical profile of Behcet's disease in a tertiary care centre in South India

N Abraham George, Ruchika Goel, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Behcet's disease is a multisystem inflammatory disorder with diverse clinical manifestations. There is a paucity of data regarding its demographic and clinical features and HLA typing.
Table 1: Clinical manifestations and treatment of Behcet's disease

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Objective: To study the demographic features, various clinical manifestations, HLA typing and treatment of patients diagnosed with Behcet's disease in patients attending rheumatology clinic in a tertiary care centre in South India.

Methods: Outpatient and inpatient charts of patients with a diagnosis of Behcet's disease were studied from our database. Patients filling ISG or ICBD criteria for behcet's disease were included. Demographic and clinical features, Lab investigations and treatment given were noted.

Results: 58 patients of behcet's disease patients were identified. Mean age at diagnosis was 33.2 years. There were 40 males (67.7%) and 19 females (32.2%). Mean duration of illness at diagnosis was 55.36 months. All patients (59, 100%) had oral ulcers. 54 (91.5%) had genital ulcers. Other manifestations were cutaneous (33, 55.9%), Ocular (26, 44%), Vascular abnormalities (20, 33.8%), Gastrointestinal (5, 0.08%), Constitutional symptoms (11, 18.6%), arthritis/arthralgia (24, 40.6%). Pathergy test was positive in 14/35, 40%) and HLA b51 was positive in 16/43, 37.2%). Mean ESR was 39.39 mm at 1 hour and mean CRP was 24.02 mg/dl. Most patients were given steroids (53, 89.8%) in various doses. Immunosuppresants or immunomodulators used were Azathioprine (30), Methotrexate (12), MMF (10), Colchicine (13), TNFis (5), Cyclophosphamide (3), HCQS(3), Cyclosporine (1), Thalidomide (1), Rituximab (1), Apremilast (2).

Conclusion: Behcets disease commonly occurs in young males. There is a huge delay in diagnosis of this disease. Oral and genital ulcers are the most common manifestations followed by cutaneous, ocular, musculoskeletal, vascular and constitutional features. Sensitivity of pathergy test and HLA B51 was 40% and 37.2% respectively. Steroids are used in most patients and azathioprine was the most often used immunosuppressant.


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To study the clinical, laboratory profiles and outcomes of patients presenting with digital gangrene to a rheumatology clinic in a tertiary care hospital

Kriti Kishor, Prasanna Dogga, Kunal Chandwar, Digvijay Ekbote, Juhi Dixit, Puneet Kumar, Urmila Dhakad; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Gangrene is a life threatening manifestation of various autoimmune disorders often leading to digit or limb loss, mandating early recognition.

Objectives: To study the clinical, laboratory profile and outcomes of digital gangrene in patients presenting to Rheumatology clinic in a tertiary care hospital.

Methods: This prospective observational cohort study was conducted in the Clinical Immunology and Rheumatology department of a tertiary care hospital in Northern India over a period of 24 months. Clinical details and various outcomes assessed at 1 month, 3 months and 6 months follow up.
Table 1: Baseline clinical, demographic and laboratory characteristics of the study population

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Table 2: Depicting various clinical outcomes at 1, 3 and 6 months follow up

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Results: A total of 64 patients were recruited. 49 (76.56%) patients were found to have an autoimmune aetiology and were followed up. Most cases of digital gangrene were seen with primary Vasculitides (n=14; 28.5 %) followed by SSc (n=11; 22.5%), SLE (n=11; 22.5%) and others (n=13; 26.5%) including primary APS (n =4), rheumatoid arthritis (n=4), infection associated vasculitis (n=3) and 1 patient each of primary Sjogren's syndrome and Cryofibrinogenemia. Pre-gangrene of digits was present in 51.02% (n=25). 43 patients had (87.75%) had multi-digital involvement. Dorsalis pedis (32.7%) and anterior tibial artery (32.6%) were most commonly involved vessels. Contrast enhancement of involved vessel wall ± mural thickening (n=10, 20.4%) and thrombus (n=10, 12.2%) were common findings. As initial therapy 36/49 (73.47%) patients received oral glucocorticoids with 16 patients receiving pulse methylprednisolone. Cyclophosphamide was the most common steroid sparing drug used (n=26) and anti-coagulation was given in 23 patients (46.9%). At the end of 6 month follow up 8 patients had expired and 5 lost to follow up. Cardiogenic shock was the most common immediate cause of death (n=5). 16 patients (28.6%) underwent surgical amputation.

Conclusion: Gangrene etiology in autoimmune disorders needs timely recognition and early treatment. Non- rheumatological causes like atherosclerosis and tropical infections form a good proportion of digital ischemia patients referred to Rheumatology for evaluation and should be recognised early for timely referral.


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A study to review current management of giant cell arteritis patients referred to District General Hospital in Shropshire

Amol Sagdeo; Robert Jones Agnes Hunt Orthopaedic Hospitals, NHS Foundation Trust, Oswestry, UK

Background: GCA is a medical emergency and about 17% reported irreversible visual loss and 1% stroke in UK due to GCA. Time to start glucocorticoids is critical and prompt review in primary care and secondary care could prevent morbidities. We reviewed the journey of suspected GCA patients in Shropshire referred to our hospital.

Objectives: To review current practices for GCA patient management in the region. To recommend pathway for suspected GCA patient management to match with the standards as per BSR GCA guidelines.

Methods: We looked at 34 patients referred from Primary care and secondary care to rheumatology with suspected GCA between April 2019 to October 2021. Data was collected retrospectively from GP records, discharge letters from District General Hospital clinical portal and electronic patient records from our hospital [Figure 1].
Figure 1: Referral source

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Results: Male: Female - 18% versus 82%. Referral sources were SDEC (38%), Ophthalmology (24%), Inpatients (20%), direct GP (6%), ccc/ shropdoc (9%), Unknown (3%). Common presenting symptoms were Headache (59%), Scalp or Temporal artery tenderness (41%), visual disturbances (41%), Jaw claudication (18%), weight loss, night sweats (3%). Temporal artery biopsy [Figure 2] was done only in 17 (50%) patients of which, TAB was positive in 10 (58.8%), negative in 4 (23.5%) and inconclusive in 3 (17.6%) patients. Only 9 (52.9%) TAB were done within 15 days, 4 (23.4%) between 15-30 days and 4 (23.4%) >30 days. Temporal artery Doppler was only done in 3 patients within 3 days and 1 patient between 3-10 days. 7/34 patients had direct advice from rheumatology team within 3 days, most patients had contact with rheumatologist for advice between 1-3 months. 5/34 patients had rheumatology clinic review <1 week, 7/34 between 1-4 weeks, 12/34 in 1-3 months, 10/34 in >3 months.
Figure 2: Temporal artery biopsy result

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Conclusions: Current practices are outliers in context of BSR 2020 guidelines[2] due to limited rheumatology emergency slots, COVID-19 backlog and post COVID catch up, lack of funding for Doppler, lack of clear fast track pathways.


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Intensive combination therapy for patients with Kawasaki disease with large coronary artery aneurysms may improve outcomes: Our experience from a tertiary care center in North India

Ridhima Aggarwal, Archan Sil, Prabal Barman, Suprit Basu, Munish Arora Eshpuniyani, Ankur Kumar Jindal, Rakesh Kumar Pilania, Pandiarajan Vignesh, Anju Gupta, Deepti Suri, Manphool Singhal1, Surjit Singh; Department of Pediatrics, Advanced Pediatrics Centre, Allergy and Immunology Unit, Post Graduate Institute of Medical Education and Research, 1Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Kawasaki disease (KD) is a common vasculitis in childhood. Literature suggests giant coronary artery aneurysms (CAAs) generally do not regress. Our experience has shown that intensive combination therapy may result in regression of these abnormalities.

Objectives: To report the outcomes in patients with KD and large CAAs managed in Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Chandigarh, India.

Methods: Data were collated from clinic records of patients with KD with large (including 'moderate' and 'giant') CAAs being followed during period January 2018 – December 2021.

Results: We identified 26 children with KD and large CAAs [moderate (n=10); giant CAAs (n=16)]. Male to female ratio was 5.6:1. Median age at diagnosis was 2 years. Treatment was initiated at median interval of 13 days from onset of fever. Of 16 patients with giant CAAs, resolution was seen in 2 (12.5%), regression to mild CAAs was noted in 5 (31.25%) and persistence of aneurysms was seen in 9 (56.25%). Eight patients received treatment with intravenous immunoglobulin (IVIg), infliximab, cyclosporine, and steroids; 4 received treatment with IVIg and infliximab; 1 received treatment with IVIg, infliximab and steroids; and 1 received treatment with IVIg and steroids. Of the 8 patients who received all 4 drugs, 50% showed regression to mild CAAs, while 50% showed no change. The 2 patients that showed complete resolution of giant CAAs had received combination therapy with IVIg and infliximab. Of ten patients with moderate CAAs, resolution was seen in 7 and regression to mild CAAs was seen in 3. No patients had persistence of aneurysms. Three patients received treatment with IVIg, infliximab, cyclosporine, and steroid; 4 received treatment with IVIg and infliximab; 2 received treatment with IVIg and steroid; and 1 received IVIg alone.

Conclusion: Treatment protocols incorporating intensive combination therapy may be necessary for patients with KD and large CAAs at presentation.


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Kawasaki disease meandering around common gastrointestinal symptoms: A diagnostic conundrum

Munish Arora, Gummadi Anjani, Rakesh Kumar Pilania, Ankita Singh, Sathish Kumar Loganathan, Deepti Suri, Vignesh Pandiarajan, Ankur Jindal, Surjit Singh; Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Kawasaki disease (KD) is the commonest childhood vasculitis. Gastrointestinal (GI) symptoms can occasionally be the forerunner of KD and may pose a diagnostic challenge to treating physicians. There is paucity of literature on GI presentations of KD.

Aims: To evaluate children with KD who had predominant GI presentations.

Methods: We analysed case records of all children with KD with GI presentations during the period January 1994-April 2021. Diagnosis of KD was based on American Heart Association criteria.

Results: From January 1994 to April 2021, we diagnosed 1078 children with KD. Of these, 24 (19 boys; 5 girls) had a GI presentation. All had GI symptoms during acute phase of disease. Median age at diagnosis was 3.5 years (range 4 weeks-13 years). Manifestations included acute gastroenteritis (n=8); blood in stools (n=3); upper gastrointestinal bleed due to duodenal ulcer (n=1); abdominal distension, vomiting, features suggestive of subacute intestinal obstruction, intussusception (n=2); colitis (n =3); mesenteric mass (n=1), ulcers in colon (n=1); gall bladder perforation (n=1); jaundice (n=7) and acute fulminant liver failure (n=1). Delays in diagnosis ranged from 7 days-4 weeks. Twenty children responded to single dose of IVIG (2 g/Kg); 3 children required a second dose of IVIG, infliximab was given in one case. 2-D echocardiography examination revealed normal sized coronary arteries in 21 patients. One patient with acute fulminant liver failure had left main coronary artery (LMCA) aneurysm (2.8 mm; + 2.7z) and macrophage activation syndrome and succumbed to illness. Two patients with jaundice had multiple aneurysms (LMCA= 6.17 mm (+7.42); LAD = 4.68 mm (+6z); RCA= 7.5 mm (+10.63Z)) and dilated right coronary artery (4.2 mm) respectively.

Conclusions: None of the GI symptoms are part of the AHA criteria. GI presentation of KD is not uncommon and may create diagnostic confusion for the treating physician. One must not overlook the common characteristic features and laboratory criteria in presence of such unusual findings because they can help in early diagnosis and management of KD, ultimately reducing morbidity.




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Antineutrophil cytoplasmic antibody-associated vasculitis: Clinical characteristics of patients from a tertiary care hospital in Eastern India

Sonali Dey, Arghya Chattopadhyay1, Pradyot Sinhamahapatra; Department of Clinical Immunology and Rheumatology, IPGME&R and SSKM Hospital, 1Department of Rheumatology, North Bengal Medical College and Hospital, Kolkata, West Bengal, India

Background: Antineutrophil cytoplasmic antibody associated vasculitis (AAV) is a predominant small vessel vasculitis with varied clinical manifestations. Disease severity can range from mild to organ threatening manifestations, and early immunosuppressive treatment is crucial to reduce mortality and morbidity. In contrast to the Western cohort, data from epidemiological studies suggest that MPA (Microscopic polyangiitis) is much more common than GPA (Granulomatosis with polyangiitis) in China and Japan. Though there are related studies from various regions of India but there is no medical literature available from Eastern India.

Aim: To describe the clinical characteristics of patients with AAV from Eastern India.

Methods: An observational cross-sectional study of clinical features, laboratory data, radiological investigations, treatment details, Birmingham Vasculitis Activity Score (BVAS) and therapeutic outcomes in patients with AAV between August 2021 to July 2022.

Results: 36 patients were diagnosed to have AAV and was the most common phenotype (22/36). Mean (SD) age at diagnosis was 41.91 (14.17). Median time to diagnosis was 6 months. 72% of our patients were females. The most common clinical manifestations were constitutional (94%), pulmonary (83.3%), renal (80.5%) and musculoskeletal (72.2%). The median BVAS was 15.5. Cyclophosphamide (72%) or rituximab (19%) were used as induction regime followed by maintenance with azathioprine (51.6%) or rituximab (48.4%). Remission was achieved in 69.4% patients. 22% patients relapsed and the major organ involvement were ocular followed by pulmonary and renal. All patients received rituximab at relapse. Two patients succumbed to illness.

Conclusion: Majority of patients were females with GPA predominance and the age of onset similar to other Indian studies. The response to treatment was good with 69% patients in remission. ENT involvement was less in comparison to other Indian studies. Ocular involvement was the most common manifestation during relapse and sustained remission was seen in all with rituximab.


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Clinicopathological profile of patients with cutaneous vasculitis

C Anoop, P S Arul Rajamurugan, R Ramesh, S Mythili; Department of Clinical Immunology and Rheumatology, Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Vasculitides are a group of disorders characterized by inflammation of vessel wall which results in various organ manifestations. Cutaneous vasculitis occurs because of involvement of small and medium vessels. It may be primary vasculitis or secondary to systemic diseases.
Figure 1: Cutaneous vasculitis-diagnosis

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Figure 2: Presentations of cutaneous vasculitis

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Objectives: To evaluate the etiological, clinical, and pathological profile of Primary cutaneous vasculitis.

Methods: Cross sectional study of patients with biopsy-proven cutaneous vasculitis who presented at rheumatology OPD from May 2022 to July 2022. Patients with cutaneous vasculitis secondary to collagen vascular disease, neoplasia and other well, defined entities were excluded.

Results: We studied 32 patients, 17 males and 15 females. IgA vasculitis seen in 16 (50%) cases, cutaneous arteritis 7 (21.80%), cutaneous leukocytoclastic angiitis 3, urticarial vasculitis 1, cryoglobulinaemic vasculitis 1, ANCA associated vasculitis 2 and granulomatous vasculitis in 1 case. We observed palpable purpura in 22 (65.6%) cases, non-healing ulcers in 8 (12.5%), tender nodules in 5 (12.5%) urticarial lesions in 1 livedo reticularis in 1, livedo racemosa in 1 and crusted plaque in 1 case. Systemic involvement was seen in 20(62.5%) cases. Antecedent fever observed in 6, COVID infection in 1, COVID vaccination in 2 and drug exposure in 3 cases. Laboratory analysis showed anemia in 7 (21.8%), leukocytosis in 11 (34.3%), raised ESR in 20 (62.5%), raised CRP in 15 (46.8%), ANA Hep2 positivity in 5, ANCA positive in 2, cryoglobulins positive in 1, low compliments in 2 cases and transient anticardiolipin positivity in 1 case. In skin biopsy 28 patient had SVV, 4 patients had MVV. Histologically severe vasculitis seen in 3 cases [Table 1].

Conclusions: IgA vasculitis was the most common diagnosis followed by cutaneous arteritis. Palpable purpura and non-healing ulcers were the common presentations. Most had no antecedent factors. History of COVID-19 infection and vaccination were also observed. Histologically most were having small vessel vasculitis.


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ANCA associated vasculitis: Experience from a South Indian tertiary centre

Yanamadal Divya, S Rajeswari, C Balaji, C Saranya, Rashi Maheswari, Joseph T Antony, M S Kavya; Department of Clinical Immunology and Rheumatology, Sri Ramachandra Medical College, Polur, Chennai, Tamil Nadu, India

Background: ANCA-associated vasculitis is a small vessel vasculitis. There are limited studies on ANCA-associated vasculitis from South India. This study aims to assess clinical manifestations and outcomes in patients of AAV in the South Indian population.
Figure 1: Cutaneous infarcts (a and b), scleritis (c)

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Objectives: To describe the epidemiology, clinical profile and outcome in South Indian population who presented with ANCA- associated vasculitis.

Methods: This is a retrospective study, data taken from hospital records over 4 years (2017-2021) of AAV patients. Clinical manifestations, outcome measures, treatment given and response to treatment were noted.

Results: Our cohort of patients [Table 1] with AAV (n=28), (GPA-21, MPA-4, EGPA-3) were identified as per 2022 ACR/EULAR criteria. 53.5% (15) were female, 46.5% (13) were male. The Mean age at presentation was 47.6 years. The Mean BVAS 3 score was 15. C-ANCA and P- ANCA positivity were seen in 50% (14) and 36% (10) patients respectively. PR3 and MPO positivity showed by 57% (16) and 39% (11). CYC, RTX, MMF and MTX were given in 64.2% (18), 25% (7), 18% (5) and 7% (2) patients respectively. 3 patients of CYC and 2 patients of MMF showed relapse. Serious infections were noted in 11% (3), minor or recurrent infections in 17.8% (5) patients.
Table 1: Clinical phenotype and treatment characteristics (n=56)

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Table 2: Comparison of characteristics with other Behçet's disease cohorts

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Conclusion: We observed upper respiratory tract followed by lower respiratory tract involvement were the most common presenting and overall manifestations. Renal involvement often accompanies pulmonary involvement. Tumefactive lesions and vascular involvement were associated with high disease activity. Both RTX and CYC showed good response compared to MMF.


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Clinical profiling and treatment characteristics in Behcet's disease: A cross-sectional study from Karnataka Rheumatology Association

Vineeta Shobha, Ramyasri Kodali, Vijay Rao1, Abhishek Patil2, Pramod Chebbi3, R Subramanian4, Sharath Kumar5; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, 1Divisha Arthritis and Medical Center, 2Department of Rheumatology, Manipal Hospitals, 5Department of Rheumatology, Optima Rheumatology Hospital, Bengaluru, 3Department of Rheumatology, SDM College of Medical Sciences and Hospital, Dharwad, 4Department of Rheumatology, JSS Medical College, JSS Academy of Higher Education and Research, Mysore, Karnataka, India

Background: Behçet's disease (BD) has a heterogeneous and unpredictable phenotype which differs in various geographical areas.

Objective: To describe clinical phenotype and medication use in BD from Karnataka, India and compare them with large cohorts from endemic regions.

Methods: We collected clinical characteristics, prescription information and clinical course of patients clinically diagnosed as BD by the practising rheumatologists in Karnataka, India.

Results: The study included 56 patients, equal gender ratio (M:F1:1) and mean age at onset being 36 ± 12 years. The frequencies of signs/symptoms are represented in [Table 1], common ones being recurrent oral aphtosis (76.8%), genital ulcers (50%) and ocular manifestations (58.9%) [Figure 1]. Almost three-fourth patients [41/56(73.2%)] fulfilled revised International criteria for behcet's disease (rICBD) criteria whereas only half 30/56 (53.6%) fulfilled International study group (ISG) criteria. The mean score as per diagnostic (rICBD) criteria was 4.66. The median duration of symptoms until establishment of diagnosis was 58 (12.5, 108) months. The most frequently used treatments were colchicine 29/56 (51.8%) and corticosteroids 43/56 (76.8%) followed by immunosuppressants [Table 1]. Eleven patients received biologic therapy (anti TNF-α) and JAK inhibitors to treat severe organ involvement. HLAB51 was tested in 36/56 patients out of which 21/36 (58.3%) were tested positive.

Pathergy test was conducted in 19/56 patients and was positive in 12/19 (63.1%) patients. Outcomes were documented in (54/56) patients on followup, among which (37/54) 68.5% had remission, (13/54) 24.07% were in partial remission, (3/54) 5.5% had persistent and (1/54) had relapsing course [Table 2].

Conclusion: We report a similar clinical profile as other BD cohorts albeit a lesser incidence of orogenital ulcers, and an excellent response to treatment.


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Rapidly progressive renal failure due to ANCA associated vasculitis: Clinical spectrum and outcome

Atanu Pal, Tanya Bhuiya, Sourav Sadhukhan; Department of Nephrology, IPGME&R, Kolkata, West Bengal, India

Background: Newer immunosuppressive regimens (Rituximab + steroid) are coming up in recent years for treating ANCA associated vasculitis (AAV) with the aim of reducing cumulative dose and side effects of immunosuppression (EUVAS) while having same efficacy as induction agents.

Objective: To study the clinical spectrum and compare the efficacy and safety of cyclophosphamide and Rituximab.

Methodology: A single centre observational retrospective study consisting of 33 AAV patients presenting with renal dysfunction over 1.5 years. Induction therapy was given according to institutional protocol (EUVAS/RAVE).

Results: Patient characteristics: Total patients - 33 New - 30, Relapse – 3 Male: Female - 12:21. Mean age - 39.6 ± 16.9 yrs. Distribution - MPO: 16, PR3: 10, MPO + PR3: 1, ANCA + ANTI-GBM: 6. Presentation - RPRF 20, HD requiring 18, DAH 18 Systemic symptoms - Joint pain, Skin rash, Fever, Weight loss. Baseline creatinine – (5.6± 2.57) mg/dl. Treatment: EUVAS protocol - 21 new, 1 relapse RAVE protocol - 7 new, 2 relapse No induction - 2 new patients, as severe sepsis. Plasmapheresis - 17 (DAH, ANCA- ANTI GBM, HD requiring). Adverse events: EUVAS - Infections 59%, bone marrow suppression 50% ; RAVE - UTI 11% patients. Discontinuation of therapy - EUVAS: 4 new patients due to severe sepsis, bone marrow suppression and progression to ESRD. No discontinuation in Rituximab arm. Death: 7. All in EUVAS arm; None in RAVE arm Timing of death - 3 within 1 month, 1 at 2.5 months, 3 after 3 months. Remission: EUVAS arm (22): 9 complete remissions; 2 partial remissions, 4 early discontinuations due to ESRD, 2 completed course but progressed to ESRD. RAVE arm (9): All but 1 had complete remission.
Figure 1: Treatment outcome in patients with IIM (%) Total improvement score (TIS) at 3, 6 and 9th month were- 38.93 + 25.59, 54.74 + 26.58 and 70.69 + 23.21 respectively

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Conclusion: Rituximab use in AAV is effective and safe.


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Treatment outcomes of patients with juvenile and adult inflammatory myositis

Arun Baby, Phani Kumar Devarasetti, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Inflammatory disorders of the skeletal muscle include dermatomyositis (DM), polymyositis (PM) and other subsets. The IMACS developed the 2016 ACR/ EULAR Criteria for Minimal, Moderate, and Major clinical response, which gives assessment of improvement.

Objectives: Outcome of treatment of patients with juvenile and adult inflammatory myositis, Parameter affecting response to treatment: Age at onset, gender, time interval between onset of symptoms and seeking medical care, type of myositis as per classification, antibodies. Frequency of involvement of extra-muscular domains.

Methods: All patients fulfilling the eligibility criteria, the 2017 ACR/ EULAR classification criteria presenting here between (May 2021 - Jan 2022) age from 5 - 70 were included in this study. Age at onset, gender, time interval between onset of symptoms and seeking, medical care was recorded. Type of inflammatory myositis subgroups, associated, antibodies frequency of various muscular and extra muscular global assessment was taken. Treatment response using 2016 ACR/EULAR criteria along with no improvement, death was noted.

Results: Our study had 23 patients, of which were 21 adults and 2 juveniles. Mean age (SD) of the adults was 36.67 +11.1 years, mean age of onset of the disease 33.65+ 12.712 months [Table 1] and [Figure 1].
Table 1: Organ involvement

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Conclusion: Majority of patient [Table 1] (52.7%) had major improvement. No association could be found between the various parameters. In extra muscular manifestation of IIM, the most common was cutaneous and least pulmonary.


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Clinical profile and role of anti-Ro52 antibody in patients with antisynthetase syndrome: Experience from a tertiary care center in South India

M Bhuvanesh, S Rajesh, Abida Aliyar; Department of Rheumatology and Clinical Immunology, KIMSHEALTH, Thiruvananthapuram, Kerala, India

Background: Antisynthetase syndrome (ASS) is characterized by presence of myositis, arthritis, interstitial lung disease (ILD), raynauds phenomenon and fever. Anti-Jo-1 is the most common antibody associated with ASS. Though Anti-Ro52 antibodies have been commonly described, the exact association in the disease manifestations remain unknown.

Objectives: To describe the clinical profile and investigate the role of anti-Ro52 antibody in the clinical manifestation of ASS.

Methods: 27 patients with ASS (20 females, mean age – 39 years) were included in the study. Patients were classified into two groups based on the positivity status of anti-Ro52 antibody. Clinical disease manifestations were compared between the two groups.

Results: The most common clinical manifestation was arthritis (17 patients) followed by ILD (15 patients) [Table 1]. Non-specific interstitial pneumonia (NSIP) was the most common pattern of ILD. Clinical myositis was seen in only 6 patients, although a total of 14 patients had creatinine phosphokinase (CPK) levels more than twice the upper limit of normal. The most common antibody was anti-Jo-1 (21 patients) followed by anti-Ro52 (16 patients). Other antibodies positive were anti-PL-7 (4 patients), anti-PL-12 (3 patients). Majority of patients (6 out of 7) with non-Jo-1 antibody were positive for anti-Ro52. Anti-Ro52 was strongly associated with presence of arthritis and ILD (p = 0.002 and 0.0015 respectively). Clinical myositis or an elevated CPK was seen more frequently in the anti-Ro-52 negative group (r=0.57, p = 0.005).
Table 1: Clinical and demographic characteristics of individuals with anti-MDA-5 antibody dermatomyositis

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Table 2: Logistic regression analysis predicting the mortality in anti-MDA5 dermatomyositis

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Conclusions: Seronegative non erosive arthritis is the most common clinical manifestation. Anti-Ro52 antibody is associated with development of arthritis and ILD; and may even have a protective role against myositis. Studies with larger numbers and longer follow up are needed to confirm the role of anti-Ro52 antibody in ASS.


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Clinical characteristics, therapeutics and treatment outcomes of adult patients with anti-MDA5 dermatomyositis: A single centre experience from South India

Shivraj Padiyar, Aswin M Nair, Bijesh Yadav, Prathyusha Manikuppam, Avanish Jha, Abhilasha Manwatkar, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Literature reported on anti-MDA5 dermatomyositis from the Indian Subcontinent is scarce due to the rarity of presentation and inadequate identification.

Objectives: To study the demographic, clinical characteristics, and treatment outcomes of patients with anti-MDA5 dermatomyositis.

Methods: This is a retrospective study done between 2019 and 2021 in a tertiary care centre from South India. All patients, presenting to the Rheumatology department, classified as idiopathic inflammatory myositis (IIM), and positive for anti-MDA5 antibody were included in the study. Baseline characteristics of anti-MDA5 patients were compared with the data of non-MDA5 patients over the last 10 years. Clinical, biochemical, and treatment responses were assessed on follow-up. Complete and partial responders were identified using predefined criteria. Factors predicting mortality were determined by logistic regression analysis.

Results: A total of 29 adult patients of IIM were positive for anti-MDA5 antibody during the study period. The mean (±SD) age of the patients was 40.3 (±13.02) years with the female: male ratio of 1.4:1. Gottron's sign (p<0.001), panniculitis (p<0.001), calcinosis (p<0.001), cutaneous ulcerations (p<0.001), inflammatory arthritis (p<0.001) and ILD (p<0.02) were present more commonly in the anti-MDA 5 IIM group, whereas myopathy (p<0.001), elevated CPK (p<0.001), and LDH (p<0.001) were more frequently present in the non-Anti-MDA 5 IIM group (N=421) [Table 1]. Complete response was seen in 10 patients (43.4%), while partial response was seen in 8 patients (34.7%). Five patients died on follow-up, accounting for a mortality of 21%. Age >50 years was significantly associated with mortality [p=0.025, [Table 2]].
Table 1: Baseline characteristics of patients

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Conclusion: Anti-MDA5 dermatomyositis represents a distinct and unique subset of Idiopathic inflammatory myositis with characteristic clinical manifestations. A combination of high-dose steroids with mycophenolate led to improvement in all the patients with anti-MDA5 antibodies with non-RP-ILD presentations. Elderly age is a poor prognostic factor of mortality.


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Primary Sjogren's syndrome in children: An experience from a single center

Debasis Patro1, B Lakshminarayan2, Indhuja Rajarathinam2, Jyothi Raghuram2,3, Anand Prahalad Rao1,2; 1Department of Rheumatology, Manipal Hospital, 2Department of Rheumatology, Indira Gandhi Institute of Child Health, 3Department of Rheumatology, Aster Hospital, Whitefield, Bengaluru, Karnataka, India

Introduction: Primary Sjogren's Syndrome (pSS) is a rare disorder often known as autoimmune exocrinopathy or epithelitis. Unlike in adults, it does not present with sicca-like manifestations in children which leads to a delay in diagnosis.

Objective: To describe the varied clinical manifestations of pediatric Sjogren in our cohort. To assess the EULAR Sjogren's Syndrome Disease Activity Index (ESSDAI) at the time of diagnosis.

Methods: Retrospective analysis of case records of 9 patients of pSS fulfilling the 2016 ACR- EULAR Classification criteria and Proposed Juvenile pSS by Bartunkova et al.

Results: In our series, the majority were females (M: F1:8.9). The mean age of presentation was 11.5 years (±2.45 years). The findings are summarized in the table below. Extra-glandular manifestations were more common at presentation. 66.7% of patients had arthralgia/arthritis followed by manifestations due to renal tubular acidosis (RTA) in 44.4%. 2 patients each initially presented with Immune-mediated thrombocytopenia (ITP) and cutaneous vasculitis. The mean ESSDAI score at onset was 6.88 ± 1.98. The most frequently scored domain in ESSDAI was constitutional followed by the articular and glandular domain. Activity level was more in the articular and hematological domains. The limitation of our study was the lack of demonstration of objective involvement of the salivary gland.

Conclusion: Sjogren's should be kept in the differential of U-CTD with positive surrogate markers in a resource-poor setting which helps bridge the gap between diagnosis and timely interventional. ESSDAI score at the onset with involvement of multiple domains guides us for aggressive immunosuppression.


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The association of myositis specific antibodies in North Indian patients with inflammatory myositis

Shahzeene Dhuria, Shounak Ghosh, Prabin Khatri, J N Durga Rao Yadavalli, Kaustubh P Telang, Vinay Singal, Rajiva Gupta, Gargi Singh, Abhinav Yadav, Rehyan Khan; Department of Rheumatology and Clinical Immunology, Medanta-The Medicity, Gurugram, Haryana, India

Background: Studies in Autoimmune Inflammatory Myositis (AIM) have shown that certain antibodies have a role in the diagnosis and prognosis of patients with myositis. This ongoing study presents the preliminary data of 132 patients with AIM from a North Indian tertiary care center.

Objectives: To study the prevalence of Myositis specific antibodies (MSA) and Myositis Associated antibodies (MAA) in Indian patients with AIM and correlation of these antibodies with clinical features.

Methods: All consecutive patients with AIM (satisfying the Bohan and Peter criteria, 1975) attending the Rheumatology and Clinical Immunology department of Medanta Hospital, Gurugram from November 2016 to August 2022 were included prospectively and divided into groups as Dermatomyositis (DM), Polymyositis (PM), CTD associated myositis (CTD-M), Cancer associated myositis (CAM) and Juvenile Myositis (JM). The study was approved by the Institutional Ethics Committee. Patients' clinical data and sera were collected and analysed after informed consent.
Figure 1: Box and Whisker plot of HAQ-DI score in fatigue and non-fatigue patients

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Results: The cohort consisted of 132 patients with a mean age of 45.8 years. Fifty of them were DM, 48 were PM, 21 were CTD-M, 6 were CAM and 7 were JM. 69.20% were ANA positive, 22 % had MSA, 26.80% had MAA and 10.60% had both. Presence of antibodies against Mi-2 and Jo-1 was found in 13 (32.50%) patients each, PL-7 in 3 (7.50%), PL- 12 in 4 (10%), SRP in 8 (20%), MDA-5 and NXP2 in 1 (2%) patient each. Antibodies against Ro, RNP were present in 7 (15.22%) each and PM-Scl in 9 (19.57%) of the patients. Mi-2 antibodies were mainly found in patients with DM and was significantly associated with rash. ILD was associated with Jo-1 and Ro antibodies.

Conclusion: MSA was seen in 22 % and MAA in 26.80%. Mi-2 antibodies were associated with rash and none had ILD whereas Jo-1 antibodies were associated with mechanic hands, arthritis and ILD. With further recruitment of patients in this ongoing study, we hope to obtain more robust data in future.


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Prevalence of fatigue and its impact on quality of life in the patients with primary Sjogren's syndrome

Diganta Das, Prateek Deo, Jithin Methew, Shankar Jayaprakash, G S R S N K Naidu, Sanjay Jain, Sandeep Grover1, Aman Sharma, Varun Dhir, Shefali Khanna Sharma; Departments of Internal Medicine, Rheumatology Wing, PGIMER, 1Department of Psychiatry, PGIMER, Chandigarh, India

Background: Among the extra-glandular symptoms in primary Sjogren's syndrome, fatigue and pain are the most common. Fatigue is a poorly understood phenomenon with multi-faceted involvement. Therefore, it is important to assess the severity of fatigue and describe its various dimensions while assessing fatigue. On the other hand, fatigue is also one of the predictors of reduced health and quality of life in pSS.

Objectives: To assess fatigue by FSS and Profile of fatigue questionnaires and quality of life with HAQ-DI and to know the association between the two in patients with PSS.
Table 1: Demographic and disease manifestations data

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Methods: We enrolled 125 patients according to the AECG 2002 and ACR-EULAR 2016 criteria for the classification of Primary Sjogren's Syndrome (pSS). Fatigue was assessed with the Fatigue Severity Scale (FSS) and the Profile of Fatigue (ProF). The quality of health was assessed with HAQ-DI scale. Associations with fatigue was compared using multivariate regression analysis.

Results: Fifty-seven (45.6%) patients had fatigue in this study cohort. Among the patients with fatigue, 12 (21%) patients scored less than 4 on fatigue severity scale, 36 (63.1%) had mild fatigue, 7 (12.2%) had moderate fatigue and only 2 (3.5%) patients had severe fatigue. The mean FSS score in the cohort was 2.9 ± 1.5. The ProF mean scores were 2.7 ± 0.6 for somatic fatigue and 2.9 ± 1.3 for mental fatigue. There was a significant difference between the median HAQ-DI scores in fatigue and non-fatigue groups. There was a positive correlation between fatigue score and HAQ-DI score. The prevalence of depression and somatoform disorder were 15.2% and 28% respectively. The ESSPRI, ProFAD Ocular and Oral dryness and PHQ-9 score positively associated with higher fatigue score.

Conclusions: The pSS patients with fatigue had higher disability and impaired quality of life. The pain, dryness and presence of depression are the predictors of fatigue in patients with pSS.


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A retrospective analysis of autoantibody profile of systemic sclerosis patients in a tertiary care centre

Nagamounika Kothapalli, Josna Joseph, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvasculopathy, immune disregulation, visceral and cutaneous fibrosis. Autoantibodies directed to multiple intracellular antigens are present in more than 95% of SSc patients. They are associated with distinctive clinical subsets and specific patterns of organ involvement.[2]

Objective: To study the clinical manifestations and various autoantibodies in systemic sclerosis patients.

Methods: A retrospective analysis of autoantibody profile in SSc patients, fulfilling ACR/EULAR classification criteria and for whom line immunoblot (LIA) (EuroimmuneSSc profile) data was available were chosen for this study. Demographic data, disease duration, ANA specificities,various organ manifestations and autoantibody profile in LIA were collected for a period January 2016 – May 2022.

Results: Out of the 233 samples for whom SSc profile testing was carried out, 86 patients fulfilled ACR/EULAR criteria for SSc. Demographic data and disease manifestations are summarised in [Table 1]. ANA by indirect immunofluorescence showed 93.8% positivity (76/81). In 5 ANA negative patients 4 ha Ro 52 positivity. Among the autoantibodies in SSc, anti-topoisomerase 1 (anti-Scl-70) was the most prevalent, seen in 42 (48.8%) patients, Ro 52 was the second most prevalent seen in 25 (29%) of patients. CENP was positive in 8 (80%) of the limited SSc patients. Various clinical manifestations and associated antibody prevalence is summarised in [Table 1]. Three patients had scleroderma renal crisis and 2(66.6%) had RNA polymerase 3 positivity and 1 (33.3%) had U3RNP positivity.

Conclusion: Autoantibody profiling aids in early and accurate diagnosis of SSc, and can be possibly linked with distinctive clinical phenotype.


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A study on the assessment of cardiovascular risk and CIMT in patients of systemic sclerosis and correlation of CIMT with disease severity

Prasanna Dogga, Kunal Candwar, Digvijay Ekbote, Juhi Dixit, Kriti Kishor, Urmila Dhakad, Puneet Kumar; Department of Clinical Immunology and Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Accelerated atherosclerosis is well-known in CTD however is less reported in Ssc.

Objective: To study the Cardiovascular risk (QRISK 3) and CIMT in patients of Ssc and to correlate the CIMT of patients with Ssc Disease Severity score (MEDS).

Methods: Data of 72 patients including clinical, serological profile and treatment were recorded [Table 2]. CV risk by QRISK 3 calculator, CIMT by Ultrasonography are used. Correlation between Cardiovascular profile with CIMT and disease severity were seen. 34 age comparable controls were included.
Table 1: Important results of patients

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Table 2: Severtiy of PAH

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Results: A total of 72 SSc patients (lcSSc -27, dcSSc -45). F> M (11:1). The median duration of disease enrolled was 2.9 years and time to referral to a specialist was 1.28 years. Lipid profile showed low HDL, high LDL, TG, Cholesterol as compared to controls [Table 1]. The mean CIMT of the patients showed a significant difference when compared to controls (0.58 ± 0.09 vs. 0.56 ± 0.10; p=<0.01). The mean mRSS was 23.99 ± 10.44 and mean MEDS score was 8.68 ± 2.76. Forty-three patients (59.7%) out of 72 had ILD documented by HRCT. NSIP in 22 (30.6%) and UIP in 21 (29.2%) patterns observed. Severity of PAH (Mpap>20 mmHg) is greater in lcSSc vs dcSSc (30.33 ± 10.50 vs. 28.42 ± 7.38) [Table 2]. Cyclophosphamide, MMF were used for ILD, skin. The mean CIMT (0.58 ± 0.09) showed a significant positive correlation with QRISK3 however it is weak with rho=0.575, p value <0.001. The mean CIMT (0.58 ± 0.09 mm) showed a negative correlation with mRSS with rho = -0.145 and p value 0.224 [Figure 1].
Figure 1: Kaplan Meier survival curve for patients with Ssc and cardiac involvement with follow up

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Conclusion: CIMT had positive correlation with PAH (significant, p=0.034) and QRISK3 (significant, p=<0.01). CIMT had negative correlation with mRSS. The mean CIMT showed no correlation with MEDS score.


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Clinical profile and outcome of patients with cardiac involvement in systemic sclerosis

Ravi Kumar, Ruchika Goel, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Primary cardiac disease is relatively uncommon in systemic sclerosis (SSc) and is associated with adverse prognosis. The prevalence of primary cardiac involvement in SSc ranges from 15–30%. There is paucity of systematic studies addressing cardiac involvement in Indian patients with SSc.

Objectives: The current study aimed to describe the spectrum and 3-year survival rate of patients who were diagnosed with systemic sclerosis with cardiac involvement.

Methods: Electronic medical records of all patients who attended our clinics and were diagnosed with systemic sclerosis between January 2017 and July 2022 were screened. Consecutive patients with cardiac involvement with or without pulmonary hypertension were included in this retrospective analysis. Patient with only pulmonary hypertension without endo-myocardial involvement were excluded. Data regarding clinical presentation, laboratory tests, electrocardiography, echocardiography, Holter monitoring, cardiac imaging, management and follow up were noted. The survival data patients who were lost to follow up was collected telephonically.

Results: Among 102 patients with SSc screened, 19 (18.6%) had cardiac involvement with 84 (82%) patients being females. Median age at onset of SSc and diagnosis of cardiac involvement was 37.1 (Interquartile range (IQR) 24- 50) years and 41.05 (IQR 32- 50) years respectively. Median modified Rodnan skin score was 28 (IQR 8-41). Overall, 12 (11.7%) patients had isolated cardiac involvement without PAH while 7 (6.8%) had concomitant PAH. The cardiac abnormalities included LV systolic dysfunction in 11 (10.7%), pericardial effusion in 6 (5.8%), conduction abnormalities in 5 (4.9%), cardiomyopathy and arrhythmias in 2 (1.9%) each. Majority of patients (84.2 %) tested positive for anti-Scl-70 antibody while 10.5% and 5.2% were positive for anti-centromere and anti-PM/Scl-100 respectively. The demography, extra-cardiac organ involvement and autoantibody profile of patients with cardiac involvement did not differ significantly from the patients without cardiac involvement. Eleven (57%) patients had followed up physically for consultation while follow up data for 4 (21%) patients were collected telephonically. Two patients died within 7 days after cardiac diagnosis while 2 others died at 22 and 34 months respectively while 4 patients were lost to follow up. The 3-year survival was 73.3% (11/15) in this cohort [Figure 1].
Figure 1: Kaplan Meier survival curve for patients with Ssc and cardiac involvement with follow up

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Conclusion: Cardiac involvement was observed in 18.6% of our patients with SSc and was associated with mortality in 26.6%.


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Possible relationships between demyelinating disorders of the central nervous system, aquaporins, and Sjögren's syndrome: A literature review

Pulukool Sandhya, Tetsuya Akaishi1, Kazuo Fujihara1, Masashi Aoki1; Department of Rheumatology, St. Stephen's Hospital, New Delhi, India, 1Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan

Background: Primary Sjögren's syndrome (SS) is associated with neuromyelitis optica spectrum disorders (NMOSD), a demyelinating autoimmune disorder. Intriguingly, osmotic demyelination syndrome (ODS), a non-inflammatory disorder has been reported in the context of SS with renal tubular acidosis (RTA) that are not established risk factors for ODS.

Methods: A literature search was undertaken to identify ODS in patients with both SS and RTA (SS-RTA) and clinical and laboratory features were compiled. We also screened for ODS in SS (without RTA) and RTA (without SS) as well as NMOSD in SS-RTA.

Results and Discussion: We identified 14 patients (all women, median age 41 years) with ODS amongst 457 patients with SS-RTA. Twelve had quadriparesis and ten patients had one of the following features either alone or in combination namely worsening of the sensorium, extensor plantar response, dysphagia/dysarthria and facial palsy. Ocular palsy, a late manifestation was seen in four patients. One patient with long segment myelitis and subsequent ODS died. Most patients recovered without significant neurological sequelae. None had hyponatremia while all patients had hypokalemia and/or hypernatremia. Hypokalemia causing nephrogenic diabetes insipidus (NDI) and subsequent hypernatremia resulting in osmotic stress could potentially explain the occurrence of ODS. Defective aquaporin (AQP) is a common link underlying all connected diseases namely SS, NDI and ODS, raising the possibility of immune-mediated AQP dysfunction in the pathogenesis [Figure 1]. ODS was identified in 3 patients with RTA (no SS) and none with SS (no RTA) highlighting the important roles played by both SS and RTA in ODS. NMOSD was found in 4 out of 457 patients with SS-RTA Unlike ODS, NMOSD does not require a prolonged severe metabolic abnormality.
Figure 1: Possible mechanisms of ODS in SS-RTA

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Figure 1: Possible Mechanisms of ODS in SS-RTA

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Conclusion: This analysis suggests a hitherto unreported association of ODS in SS-RTA and suggests that the co-occurrence of these diseases is unlikely to be coincidental.


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Exploratory clinical subgroup clustering in systemic sclerosis patients: Results from the Indian progressive systemic sclerosis registry

Shery Susan Philip, Ramya Janardana, Revanesh S Mirji, Chengappa Kavadichanda1, Devender Bairwa1, Geetabali Sircar2, Parasar Ghosh2, Anupam Wakhlu3, Padmanabha Shenoy4, Sumithra Selvam5, Vineeta Shobha for Indian Progressive Systemic Sclerosis Registry; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, 5Division of Epidemiology and Biostatistics, St. John's Research Institute, Bengaluru, Karnataka, 1Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 2Department of Rheumatology and Clinical Immunology, Institute of Post-Graduate Medical Education and Research and S. S. K. M. Hospital, Kolkata, West Bengal, 3Department of Clinical Immunology and Rheumatology, Apollo Medics Super Speciality Hospitals, Lucknow, Uttar Pradesh, 4Centre for Arthritis and Rheumatism Excellence, Kochi, Kerala, India

Background: Classification of systemic sclerosis (SSc) into subsets is challenging due to its heterogeneity.

Objectives: To identify SSc subsets based on antibody and clinical characteristics in the Indian population.

Methods: We included 430 SSc patients satisfying ACR-EULAR classification criteria from the multicentric IPSSR between 2019-21. Two-step cluster analysis (unsupervised) was performed to identify subgroups based on immunological and clinical characteristics.

Results: We differentiated 4 clusters based on autoantibody and clinical features. Baseline characteristics are described in [Table 1], and distribution of clinical characteristics and autoantibodies across clusters in [Figure 1]. Cluster-1 had high prevalence of anti-centromere antibody (ACA) and clusters 2, 3 and 4 had anti-topoisomerase (topoI). Amongst topoI positive clusters, cluster -2 had limited skin pattern, while clusters 3 and 4 had diffuse skin disease. Modified Rodnan skin score (MRSS) differed significantly between topoI clusters. Cluster-4 had younger age at disease onset (p<0.0001), diffuse skin pattern, large joint contractures, tendon friction rubs, and highest prevalence of musculoskeletal features, GI features and vasculopathic features. Cluster-4 had the worst MRSS (p<0.0001), patient and physician global assessments (p<0.0001) and BMI (p=0.018), indicating a severe subset. Cluster-1 represented a mild subset with older age at onset (p<0.0001), limited skin pattern, and least MRSS (p<0.0001). The prevalence of interstitial lung disease (ILD) was similar in all 3 topoI positive clusters, indicating strong association of topoI with ILD irrespective of extent of skin involvement.
Table 1: Baseline characteristics used in cluster analysis (n=430)

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Conclusion: We identified 4 clusters, 3 with topoI positivity, reflecting a spectrum of cutaneous severity and organ involvement that may help in risk stratification. TopoI was strongly associated with ILD irrespective of extent of skin involvement. Cluster-4 represented the most severe disease in terms of MRSS, global assessments and clinical features.


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Semi-quantitative thigh magnetic resonance imaging scores in assessing disease activity and determining long-term clinical outcome in idiopathic inflammatory myopathies: A causal mediation analysis

Mamatha Gorijavolu, Devender Bairwa1, Chengappa G Kavadichanda, Sachit Ganapathy2, Saikumar Dunga, Aishwarya Gopal, Ramesh Ananthakrishnan3, Molly Mary Thabah, Vir Singh Negi4; Departments of Clinical Immunology, 2Biostatistics and 3Radiology, JIPMER, Puducherry, Departments of 1Medicine and 4Clinical Immunology, All India Institute of Medical Sciences, Bilaspur, Himachal Pradesh, India

Background: tMRI which is a commonly used modality in the evaluation of IIM has not been explored as a marker of long-term outcome.

Objectives: To evaluate the relationship of baseline t-MRI scores with muscle strength, muscle enzymes and autoantibodies and to determine the demographic, clinical and serological factors mediating t-MRI changes which determine poor long-term skeletal muscle outcome.

Methods: This is a retrospective analysis of a single-centre myositis cohort. Patients (n=59) with tMRI at baseline were included. MRI images were scored for intramuscular and fascial edema, muscle atrophy and fatty infiltration. On follow-up, the maximum MMT-8 was recorded for patients in remission (n=38). Spearman correlation was done between clinical parameters, muscle enzymes and tMRI scores. Causal mediation analysis was carried out to determine the effect of sociodemographic, clinical, and serological variables on follow-up MMT8 score through mediating tMRI changes.

Results: Baseline MMT-8 had significant negative correlation (p<0.05) with muscle edema (r=-0.755), fascial edema (r=-0.443) and muscle atrophy (r=-0.343). Maximum MMT-8 showed significant negative correlation with muscle atrophy (r=-0.497, p=0.001) and fatty infiltration (r=-0.531, p=0001) [Figure 1]. Association of tMRI scores with autoantibodies is depicted in [Table 1]. Male sex had positive total effect on follow-up MMT-8 due to less atrophy (2.75 [0.24, 4.98]; p =0.028) and fatty infiltration (2.15 [0.45, 3.91]; p = 0.014), higher age had negative total effect due to more atrophy (-0.10 [-0.19, 0.01]; p =0.036) and fatty infiltration (-0.07 [-0.15, 0.00]; p = 0.042), Antisynthetase antibody positivity had positive total effect due to less fatty infiltration (3.63 [0.79, 5.77]; p = 0.010) and longer disease duration had negative total effect due to higher fatty infiltration (-0.17 [-0.26, 0.02]; p = 0.038).
Figure 1: Correlation of tMRI scores with baseline and follow-up MMT-8

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Table 1: Association of baseline thigh magnetic resonance imaging scores with clinical and antibody -based subgroups

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Conclusions: Baseline fatty infiltration and muscle atrophy determine eventual skeletal muscle recovery. Higher age, female sex, absence of anti-synthetase antibodies and longer disease duration cause muscle damage to some extent but the other pathways of muscle damage remain unexplored.


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Effect of atorvastatin on vascular endothelial function in early systemic sclerosis: An open label randomized controlled trial

Anna C Das, Molly Mary Thabah, V S Negi1, Christina Mary Mariaselvam, Avinash Anandaraj, Rubini Vijayan2; Departments of Clinical Immunology and 2Cardiology, JIPMER, Puducherry, 1Department of General Medicine, AIIMS, Bilaspur, Himachal Pradesh, India

Background: Role of statins in tackling micro vascular dysfunction in established systemic sclerosis has been explored in case series and placebo controlled trials with conflicting results.

Objectives: To assess effect of atorvastatin 40 mg on endothelial function in early systemic sclerosis.

Methods: Systemic sclerosis patients (18 -65 years), having disease duration less than 3 years from the first non-Raynaud's symptom, classified by ACR-EULAR 2013 criteria were enrolled. Those with diabetes, hypertension, heart disease, recent (<4 weeks) dose change of calcium channel blockers/ACE inhibitors, digital gangrene and overlap syndromes were excluded. Participants were randomized to the treatment (StatinA40) arm or control arm using block randomization in blocks of four. Sequentially numbered opaque sealed envelopes (SNOSE) method were used for allocation concealment. There was no blinding. Control arm received Immunosuppressive and recommended symptomatic treatment in stable doses for 16 weeks. Treatment arm received atorvastatin 40 mg/day in addition to above. Primary outcome measure was change in brachial artery flow mediated dilation (FMD %) at 16 weeks. Secondary outcome measures were change in Carotid intima medial thickness (CIMT) and biochemical measures (high sensitivity CRP, von Willebrand Factor, oxidized LDL). Outcome measures at 16 weeks was compared between groups using independent student t-test/Mann- Whitney U test, as baseline was comparable in both groups.

Results: Baseline characteristics were comparable in treatment and control arms. Change in Flow mediated dilatation (FMD) was comparable between groups at 16 weeks.

There was no significant change in any of the secondary outcomes at 16 weeks between two arms. There was significant difference in basal brachial artery diameter (BBAD), between groups at 16 weeks (p=0.008) [Table 1] No drug related adverse events were noted in either arm. Attrition was more in the treatment arm [Figure 1].
Figure 1: CONSORT flow diagram

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Table 1: Primary and secondary outcome in control and treatment (A40) arms

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Conclusion: In early systemic sclerosis, 16 weeks of atorvastatin 40 mg/day, did not significantly change FMD in either arm. Endothelial function evaluation using FMD might not be sufficient to demonstrate significant change without large number of subjects.


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Clinical profile and outcome of ILD associated with systemic sclerosis from a tertiary care center in North India

Anu Balakrishnan, Sarit Sekhar, Sakir Ahmed, Durga Prasanna Misra, Vikas Agarwal; Department of Clinical Immunology and Rheumatology, SGPGIMS, Lucknow, Uttar Pradesh, India

Introduction: Systemic sclerosis is associated with significant morbidity and poor survival. ILD is one of the predominant manifestation and the reason for poor survival. The outcome of patients with scleroderma ILD and predictors of mortality is assessed in this study.

Methods: A retrospective study was conducted in which patients who were registered in our clinic from 2010-2015 were recruited. Their clinical history, and details on admission were collected form hospital records. Survival analysis and predictors of mortality was assessed in these patients.

Results: We had a total of 261 patients who were diagnosed with scleroderma during 2010-2015. Out of 261, 113 (42.53%) had ILD, 46 (17.62%) had PAH, 16 (6.13%) had cardiac disease (first- and second-degree heart blocks and diastolic dysfunction), and 8 (3.07%) had SRC. Mortality in our cohort was 90 per 1000 patient years. Infections were the most common cause of mortality in 45%. Infections and cardiac disease were the predictors of mortality with HR of 5.99 (1.68-21.49) and 7.55 (1.2-47.36) respectively in this cohort. No predictors for disease progression was identified.

Conclusion: Infections and cardiac disease were the predictors of mortality in our cohort.


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A comparative study of modulatory interaction between cytokines and apoptotic proteins among scleroderma patients with and without pulmonary involvement: Report from Western India

Vandana D Pradhan, Prasad V Khadilkar, Vidya D Kharkar1, Uday S Khopkar1, Milind Y Nadkar2, Anjali G Rajadhyaksha2, Seema H Kini3, Manisha R Madkaikar4, Durga A Chougule, Tanaya Tipnis; Departments of Clinical and Experimental Immunology and 4ICMR-National Institute of Immunohaematology, KEM Hospital Campus, 1Department of Skin, STD and Leprosy, Seth GS Medical College and KEM Hospital, 2Department of Medicine, Seth GS Medical College and KEM Hospital, 3Department of Medicine, TNMC and BYLN, Mumbai, Maharashtra, India

Background: The pathogenesis of scleroderma (Systemic sclerosis [SSc]) is associated with endothelial injury or tissue fibrosis caused by imbalance of cytokines and defective apoptosis. Pulmonary involvement is considered a major cause of mortality among these patients.

Objective: To investigate the interaction between cytokines and apoptotic proteins in scleroderma patients with and without pulmonary involvement.

Methods: Treatment naïve newly diagnosed Scleroderma patients (n=100) fulfilling ACR/EULAR 2013 criteria for Systemic Sclerosis and healthy controls (n=100) were enrolled in this study over a period of three years. Serum cytokines were assessed using bead based flow cytometry assay while the apoptotic proteins were evaluated by ELISA. Autoantibodies profile was assessed qualitatively by ANA Scleroderma dot blot. The severity of skin thickening was evaluated by modified Rodnan skin score (mRSS).

Results: Thirty eighty patients were diagnosed with pulmonary manifestations where 12% had Pulmonary Arterial Hypertension (PAH) and 26% had Interstitial Lung Disease (ILD). Serum levels of TNFα, IL-4, IL-1β, TGF-β2 and TGF-β3 were significantly elevated while IL-22 and TGF-β1 were significantly reduced among scleroderma patients when compared to healthy controls (p<0.05). The apoptotic proteins granzyme B and perforin levels were found to be significantly elevated among scleroderma patients in comparison to healthy controls (p<0.05). Serum TGF-β2 were significantly elevated among scleroderma patients with pulmonary manifestations (165.0±77.6 pg/ml) when compared with scleroderma patients without pulmonary manifestations (137.9±67.4 pg/ml) (p=0.031). TGF-β1 correlated positively with caspases (1, 8 and 9) among patients with pulmonary manifestations, whereas patients without pulmonary manifestations showed a negative correlation between TGF-β3 and caspases (1, 3 and 9) (p<0.05). The skin thickening severity positively correlated with TGF-β2 and TGF-β3 among patients with pulmonary manifestations (p<0.05).

Conclusion: The significant increase of TGF-β2 and its correlation with apoptotic proteins caspases among pulmonary scleroderma patients in comparison with patients without pulmonary involvement may suggest its involvement in pulmonary damage leading to fibrosis.


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Antisynthetase autoantibodies and their associated clinical phenotypes: Experience from a tertiary care center

Prabin Khatri, Shahzeene Dhuria, Shounak Ghosh, Kaustubh P Telang, Vinay Singal, Rajiva Gupta; Department of Rheumatology and Clinical Immunology, Medanta-The Medicity, Gurugram, Haryana, India

Background: Antisynthetase syndrome (ASS) is an autoimmune syndrome ssociated with autoantibodies targeting tRNA synthetases, which are considered to be myositis-specific antibodies. There are eight identified targets of these antisynthetase antibodies (ASA): Jo-1 (histidyl tRNA synthetase), PL-12 (alanyl tRNA synthetase), PL-7 (threonyl tRNA synthetase), EJ (glycyl tRNA synthetase), OJ (isoleucyl tRNA synthetase), KS (asparaginyl-tRNA synthetase), Ha (tyrosyl-tRNA synthetase) and Zo (phenylalanyl-tRNA synthetase).

Objectives: The aim of the study is to assess clinical characteristics and disease entities of patients associated with antisynthetase autoantibodies.

Methods: We performed a search of the electronic medical records and retrospectively retrieved data of a total of 6587 ENA profiles performed at Medanta- The Medicity, Gurgaon, between July 2019 and July 2022. 61 patients were found to be positive for antisynthetase autoantibodies. Results were taken as positive on a Line ImmunoAssay (LIA) if they were above the prespecified cut-off value of 6U/mL, using the Blue DiverQuantrix-ANA25 Screen IgG kit from D-tek, Belgium.

Results: The incidence of specific ASA's were: PL-12 48% (30 patients), PL-7 27% (17 patients) and Jo-1 24% (15 patients). In one patient with Systemic lupus erythematosus there was coexistence of both PL-7 and PL-12. Among these 61 patients with ASA positivity, the prevalence of idiopathic inflammatory myopathies including ASS reached 26%. Interstitial lung disease was present in 19%, systemic lupus erythematosous in 14%, ischemic stroke in 9%, various infections in 6%, malignancies in 4%, and the remaining 22% patients had other rheumatic and non-rheumatic diseases.

Conclusion: The clinical phenotypes of patients with the presence of ASA are greatly varied. These myositis “specific” antibodies were not only detected in idiopathic inflammatory myopathy or Antisynthetase syndrome in our cohort, but also other connective tissue diseases, infections, and malignancies.


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Prevalence of complement 2 deficiency in South Asian SLE patients below 25 years of age and comparison of clinical features between C2 deficient and C2 non-deficient SLE patient

Falgunkumar K Dhoreeyanee, John Mathew, Josna Joseph; Department of Rheumatology and Clinical Immunology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Early complement deficiency is a known risk factor for development of SLE. Deficiency of C1q, C2 and C4 has been described in literatures. The C2 polymorphism has been reported to be rare with a prevalence of 1:20,000 and 1:10,000 in European Caucasians and North Americans respectively.

Objective: To look for prevalence of Complement factor 2 deficiency in patient with SLE of, age less than 25 years. To determine if Complement factor 2 deficiency testing in the study group will provide clinically useful biomarker for prognosis and to look for difference in clinical, laboratory features of patients with SLE with complement factor 2 deficiency.

Methods: From Jan 2019 to August 2022, total 150 patient have been recruited. Complement levels are planned to be analyzed by ELISA method from serum. At present we are awaiting analysis of blood samples as ELISA kit is yet to arrive. Study is to be completed in next 6 weeks.


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Diagnostic accuracy of parotid gland ultrasonography in primary Sjogren's syndrome and correlation with serological markers

Ved Chaturvedi; Department of Rheumatology and Clinical Immunology, Sir Ganga Ram Hospital, New Delhi, India

Background: Presently Sjogren's syndrome (pSS) is diagnosed with clinical features like keratoconjunctivitis sicca and xerostomia. Primary Sjogren's syndrome means features of sicca symptoms without any other connective tissue disorders. Although the 2016 ACR classification criteria of (pSS) does not include parotid gland ultrasound, abnormal echogenisity can be detected in salivary gland in pSS. Use of parotid ultrasound in pSS as a diagnostic tool needs further exploration.

Methods: Parotid gland ultrasound was conducted for 20 primary Sjogren's syndrome patients with 50 healthy controls. Parotid gland echogenicity bilaterally were graded using OMERACT scoring system. Association between parotid gland ultrasound score and disease features were analyzed to find the clinical value of parotid gland ultrasound in primary Sjogren's syndrome.

Results: Ultrasound scores in the pSS group were significantly higher than those of healthy controls (P<.05). The level of diagnostic accuracy was comparable when both parotid glands were scored and correlated with disease duration (comparative images attached) [Figure 1]. Increased Doppler signals were present in three patients. Most pSS patients with positive ultrasound result have longer disease duration, dental loss and high levels of serological markers such as anti-SSA, anti-SSB, and positive rheumatoid factor.
Figure 1: Comparative ultrasound images of the parotid gland

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Conclusion: Parotid gland ultrasound with OMERACT scoring system has revealed high diagnostic value in pSS. Parotid gland ultrasound may also have relevance in assessing the disease's severity.


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High fatigue scores in patients with idiopathic inflammatory myopathies: A multigroup comparative study from the COVAD e-survey

Mrudula Joshi1, Silvia Grignaschi2,3, Lorenzo Cavagna2,3, Minchul Kim4, Naveen R5, James B. Lilleker6,7, Parikshit Sen8, Vishwesh Agarwal9, Sinan Kardes10, Jessica Day11, 12, 13, Ashima Makol14, Marcin Milchert15, Tamer A Gheita16, Babur Salim17, Tsvetelina Velikova18, Abraham Edgar Gracia- Ramos19, Ioannis Parodis20,21, Albert Selva-O'callaghan22, Elena Nikiphorou23,24, Tulika Chatterjee25, Ai Lyn Tan26,27, Miguel A Saavedra28, Samuel Katsuyuki Shinjo29, Nelly Ziade30,31, Johannes Knitza32, Masataka Kuwana33, Arvind Nune34, Oliver Distler35, Hector Chinoy36,37,38, Vikas Agarwal39, Rohit Aggarwal40, Latika Gupta41,42,43,44, COVAD Study Group; 1Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, 9Mahatma Gandhi Mission Medical College, Navi Mumbai, Maharashtra, 5,39,41Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 8Maulana Azad Medical College, New Delhi, India, 2Department of Internal Medicine and Medical Therapeutics, University of Pavia, 3Rheumatology Division, IRCCS Policlinico San Matteo Foundation, Pavia, Italy, 4,25Department of Internal Medicine, Center for Outcomes Research, University of Illinois College of Medicine Peoria, Peoria, Illinois, 14Division of Rheumatology, Mayo Clinic, Rochester, Minnesota, 40Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA, 6,36,42Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, The University of Manchester, 37National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, The University of Manchester, Manchester, 7Neurology, Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, 38Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, 23Centre for Rheumatic Diseases, King's College London, 24 Department of Rheumatology, King's College Hospital, London, 25NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, 27Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, 34Southport and Ormskirk Hospitals NHS Trust, Southport, 43Department of Rheumatology, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, 44City Hospital, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, England, United Kingdom, 10Department of Medical Ecology and Hydroclimatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, 11Department of Rheumatology, Royal Melbourne Hospital, 12Walter and Eliza Hall Institute of Medical Research, 13Department of Medical Biology, University of Melbourne, Parkville, Australia, 15Department of Internal Medicine, Rheumatology, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Szczecin, Poland, 16Department of Rheumatology, Kasr Al Ainy School of Medicine, Cairo University, Giza, Egypt, 17Department of Rheumatology, Fauji Foundation Hospital, Rawalpindi, Pakistan, 18Department of Clinical Immunology, Medical Faculty, University Hospital “Lozenetz”, Sofia University St. Kliment Ohridski, Sofia, Bulgaria, 19Department of Internal Medicine, General Hospital, National Medical Center “La Raza”, Instituto Mexicano del Seguro Social, 28Departamento de Reumatología Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, Mexico City, Mexico, 20Department of Medicine Solna, Division of Rheumatology, Karolinska Institutet and Karolinska University Hospital, Stockholm, 21Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden, 22Department of Internal Medicine, Vall D'hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, Spain, 29Division of Rheumatology, Faculdade de Medicina Universidade de Sao Paulo, Sao Paulo, Brazil, 30Department of Rheumatology, Saint-Joseph University, 31 Department of Rheumatology, Hotel-Dieu de France Hospital, Beirut, Lebanon, 32Medizinische Klinik 3-Rheumatologie und Immunologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Deutschland, 33Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 35Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

Background: The patients' perception of physical health may not always be a direct function of disease activity, especially in chronic rare diseases like idiopathic inflammatory myopathies (IIMs). The use of patient reported outcome measures (PROMs) in studies highlight patient's perception of disease (1). Unfortunately, data on fatigue scores in IIMs is limited.

Objectives: We compared fatigue VAS (visual analogue scale) scores in patients with IIMs, autoimmune diseases (AIDs) and healthy controls (HCs) and compared with PROMIS physical functions.

Methods: Data of respondents was extracted from COVAD database, a self-reported e-survey, on August 31st 2021. Fatigue VAS scores were compared between the groups using univariate and multivariate analysis. A propensity score matched (PSM) analysis was performed on 1827 subjects after adjusting for age, gender and ethnicity. Kruskal-Wallis test and Chi-square test was used for continuous and categorical variables respectively, and Bonferroni's correction was applied for post hoc analyses.

Results: Among 6988 respondents [43.8(16.2) years, 72%-females, 55%-Caucasians], overall fatigue VAS was 3.6 mm (SD:2.7). IIMs VAS was 4.8 mm (SD:2.6), AIDs 4.5 mm (SD:2.6), and HC 2.8 mm (SD:2.6) (P <0.001). Fatigue VAS was lower in IIMs than AIDs in inactive disease defined by patient's perception and “excellent” general health condition group (P <0.05) but was comparable in active disease defined by physician assessment, patient perception, general functional status, or by steroid dose. After PSM, IIMs and AIDs had comparable fatigue levels across all levels of disease activity, although fatigue discrepancies with HCs remained. In multivariate analysis lower fatigue VAS scores were related to HC (P <0.001), male gender (P <0.001), Asian and Hispanic ethnicities (P <0.001 & 0.003).

Conclusion: There is a higher prevalence of fatigue in IIMs and other AIDs patients. Females and/or Caucasians patients suffer a higher impact of disease manifestation. Therefore, these subjects should be more carefully evaluated during outpatient visits.


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Sjogren's syndrome: Retrospective analysis and outcome measures

Meera Shah, Sapan Pandya, Puja Srivastava; Department of Rheumatology, SVP Hospital, Ahmedabad, Gujarat, India

Background: Change in EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) and EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) in patients with primary Sjögren's syndrome (pSS) have been previously described but studies regarding their correlation is scarce.

Objective: Descriptive study of Primary Sjogren's patients presenting to our OPD and comparing change in ESSPRI and ESSDAI.

Methods: Retrospective analysis of data recorded from January 2018 to June 2022. Patients fulfilling 2012 WHO criteria for Sjogren's Syndrome were selected and descriptive analysis was done and we looked at how many patients had decrease or increase in ESSDAI, how many had decrease or increase in ESSPRI over follow up and if it did not corelate in individual patients.

Results: ESSPRI values at baseline were available for 39/82 patients whereas ESSPRI values at the last follow-up (mean follow up was18 months) were available for 33 patients. The majority of the patients (62.6%) had both glandular and extra glandular manifestations [Table 1]. The change in ESSPRI from baseline (6.86) to last follow-up (6.3) was not statistically significant p 0.342. Mean ESSDAI change from baseline (10.4) to last follow-up (6.7) was available for 52 patients. It was statistically significant p <0.0013. However, there was no correlation between the change in ESSPRI and the change in ESSDAI values between these patients [Table 2].
Table 1: Baseline characteristics of patient population

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Table 2: Hematological and serological parameters of patients in the study

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Table 1: Glandular involvement

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Conclusion: The demography and clinical features matched those reported from elsewhere and while there was a greater response in proportion of patients in ESSDAI than ESSPRI over follow up, the two did not corelate although numbers were small.


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Difference between Sjogren's syndrome patients presenting with sicca versus those with extraglandular involvement: Retrospective analysis

S Pandya, R Solanki, T Parikh; Department of Rheumatology, Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: There is scarce data from the subcontinent on Sjogren's syndrome patients presentations and the differences. As a referral bias, patients present with extraglandular manifestations to rheumatology OPDs.

Objective: To look at the differences between Sjogren's syndrome patients presenting to our OPD with predominant sicca and those presenting with extraglandular manifestations.

Materials and Methods: Informed consent was taken. We did a descriptive analysis of the retrospective data available on our SS patients from April 2012 to Aug 2022. We compared the two different sets of presenting patients – sicca vs extraglandular presentations (purpura/RTA with hypokalemia/recurrent parotitis or lymphadenopathy).

Results: More patients presented with extraglandular manifestations than sicca to our OPD probably due to referral bias. Compared to those with predominant sicca, these patients were younger, had increased ESSDAI and lesser co morbidities, more cytopenias and autoantibody (Ro/La) positivity.


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Clinical profile of connective tissue disease-interstitial lung disease cohort

Vaibhavi G Velangi, Abhishek Kumar, Puneet Saxena1, Gargi Sasmal, Harsh Jain, S Kartik; Departments of Immunology and Rheumatology and 1Pulmonology, Army Hospital Research and Referral, New Delhi, India

Background: Connective tissue disease (CTD) related interstitial lung disease (ILD) is highly heterogeneous and clinical manifestations can range from asymptomatic to severe debilitating dyspnea. It is also one of the most common causes of mortality in CTD. There is paucity of CTD-ILD data in Indian patients and hence this study has been undertaken.

Objectives: To study the clinical profile of CTD patients who have ILD.

Methods: The study was conducted between February to July 2022, in the department of rheumatology and pulmonary medicine of a tertiary care center in Northern India. It was a prospective observational study. Any patient of CTD (Rheumatoid arthritis (RA), Systemic lupus erythematosus (SLE), Mixed connective tissue disorder (MCTD), Systemic sclerosis (SSc), Primary Sjogrens Syndrome (PSS), polymyositis/dermatomyositis (PM/DM), diagnosed as per extant classification criteria, with ILD. The various clinical, immunological and treatment details were recorded manually and later converted into an excel sheet.

Results: A total of 48 subjects were included. The majority of the patients (n 40, 83%) in the study population were females with a mean age of 45.6 years. ILD was most commonly seen in SSc (n 17, 35.4%) patients, followed by RA (n 14, 29.1%), MCTD (n 7, 14.5%), UCTD (n 5, 10.4%) and PSS (n 4, 8.3%) The most common respiratory symptom was dyspnea seen in (n 40, 83%) of CTD-ILD patients. Among the CTD manifestations, Raynaud's phenomenon was seen in (n 40, 83%) of patients. Other commonly associated symptoms were sicca symptoms seen in (n 28, 58%] and arthritis seen in (n 27, 56%) patients. Autoantibodies with ANA by IF were positive in 33 out of non-RA (68.7%) patients. In SSC (n 8, 47%) patients were positive for Scl70 and 2 patients (11.7%) for anti-centromere antibodies. The next common CTD ILD was RA-ILD where 100% of patients were seropositive and the most common radiological pattern was NSIP. The most common conventional disease modifying agent used was mycofenolate mofetil and 5.16% of patients received anti-fibrotic agents.

Conclusion: CTD ILD carries a better prognosis than idiopathic fibrosing ILD and is a potentially reversible entity with appropriate therapy. Hence a high index of suspicion and early diagnosis is a key.


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SCLEROID: An unambiguous bedside tool in systemic sclerosis

P D Rath, Abhijeet Kr. Agrawal, Swetal Chauhan, Rahul Bisaralli, Hiren Kalyani, Akanksha Kapoor; Department of Rheumatology, Max Hospital, Saket, New Delhi, India

Background: PROM or patient reported outcome measures are tools used to detect the impact of patients ailment. Systemic sclerosis, being a multiorgan disease with varied clinical manifestations, and depending solely on physicians assessment provides far less than perfect picture of patients key concerns. Majority of the PROMs used in systemic sclerosis were merely adopted from other diseases, hence were not scleroderma specific. ScleroID questionnaire is a simple tool developed by involving the target population, hence incorporates variables central to patients daily living.

Objectives: In this study we aim to compare and correlate SCLEROID, which is a newly developed PROM for scleroderma patients with MRSS score and RAPID3 in outpatient department of tertiary care hospital.

Methods: In this prospective study we include all patients of scleroderma and collect their clinical and demographic data and also calculate their SCLEROID score, and RAPID3 scores. The patients disease variables such as MRSS (modified rodnan skin score) score, pulmonary status, current pharmacological interventions will be recorded. Patients who did not gave consent were excluded from the study.

Results: We collected data of 30 patients, after taking informed consent. Patients clinical and demographic data along with SCLEROID questionnaire was recorded. The mean age of patients in our study is 40.86 years, 70% of our patients are females. 18 patients (60%) patients had disease duration less than 5 years and 12 patients (40%) had disease duration more than 5 years. 17 patients had diffuse cutaneous SSc and 13 patients had Limited cutaneous SSc. The mean SCLEROID score is 2.460, mean MRSS score is 7.28 and mean RAPID3 score is 7.628. ScleroID on comparison with MRSS showed significant correlation. ScleroID on comparison with RAPID3 showed significant correlation (p < 0.001).

Conclusions: SCLEROID gives a good objective measure of disease status as per patients perspective and correlated well with other tools like MRSS and RAPID3.


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Comparison of efficacy of tofacitinib and adalimumab (biosimilar) in axial spondyloarthritis: A retrospective study

Shyamashis Das, Debanjali Sinha, Rudra Prosad Goswami1; Department of Rheumatology, Institute of Neurosciences, Kolkata, West Bengal, 1Department of Rheumatology, AIIMS, New Delhi, India

Background: Tofacitinib is a newly approved oral drug for axial spondyloarthritis.

Objective: To compare the efficacy of Tofacitinib with Adalimumab in radiographic and non-radiographic axial Spondyloarthritis retrospectively.

Methods: The case records of patients of Ankylosing spondylitis (AS) or non-radiographic axial spondyloarthritis (nrAxSpA) treated with tofacitinib or adalimumab were studied retrospectively at the I-NK, West Bengal from July 2021 to April 2022. Inclusion criteria: AS or nrAxSpA, with at least high disease activity (according to ASDAS criteria), not responding with adequate tolerable doses of NSAID and/or csDMARDs for ≥3 months; follow-up available for at least three months.

Exclusion Criteria: Steroid injections in sacro-iliac joints, active systemic infection, active TB infection. Interventions: Patients were treated with tofacitinib or adalimumab. Choice between these two drugs were made after physician and patient concurrence. Patients were either treated with 5 mg twice-daily tofacitinib or 40 mg S/C adalimumab (biosimilar) every two weekly for a minimum of three months.

Results: A total of 79 patients were included (mean age 38 ± 7.5 years, mean disease duration 7 ± 4.8 years, 57% males), of which 46 were treated with tofacitinib (mean age 39 ± 7.6 years, mean disease duration 6.7 ± 5 years, 54.3% males) and 33 were treated with adalimumab (mean age 37 ± 7.5 years, mean disease duration 7.5 ± 4.7 years, 60.6% males). ASDAS at baseline and at 6 months were 3.3 ± 0.6 and 1.5 ± 0.4 respectively for tofacitinib (p= <0.001) and 3.2 ± 0.5 and 1.3 ± 0.3 for adalimumab (p=<0.001) [Figure 1]. Tofacitinib group: six patients experienced elevated liver enzymes. ASDAS and BASDAI response at 0, 2, 4 and 6 months are given in [Table 1].
Figure 1: Comparative changes in disease activity with tofacitinib and adalimumab over time

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Table 1: ASDAS and BASDAI response at 0, 2, 4 and 6 months

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Conclusion: Both tofacitinib and adalimumab are effective drugs; there is numerically higher risk of transaminitis with tofacitinib, early response is higher with Tofacitinib, final response is numerically higher with adalimumab.


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Comparison between spondyloarthritis patients with and without enthesitis who attained clinically important improvement

V Abilash Krishnan, Sayan Mukherjee, Nishant Kamble, Mukesh Maurya, Urmila Dhakad, Puneet Kumar; Department of Clinical Immunology and Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Enthesitis is an important process in spondyloarthritis and can predominate the clinical picture or serve as a marker for impending flare. Patients who have attained clinically important improvement develop flare due to multiple factors. In patients with clinical enthesitis, il-23 activating resident t cells can trigger osteoproliferation, bone remodeling and inflammation. Asas described clinically important improvement as an improvement of asdas crp more than or equal to 1.1.

Objectives: (1) To compare the characteristics in spondyloarthritis patients with and without enthesitis who have attained clinically important improvement. (2) To identify differences in nsaid requirement, disease activity measures in between the said groups.

Methods: 22 patients with spondyloarthritis without enthesitis and 18 patients with past/current enthesitis who have attained clinically important improvement (as per asdas crp cutoffs) were included in the study. Questionnaire method interview was conducted and characteristics analysed.

Results: Out of 40 patients, 38 were male and both females belonged to the enthesitis group. Mean age was 29.3 years, 26 patients had peripheral arthritis (all patients in enthesitis group had peripheral arthritis). Nsaid requirement was very high in the enthesitis group (mean nsaid score was 180 in enthesitis group vs 45 in non-enthesitis group). Most common site of enthesitis was plantar fascitis (12 out of 18 patients had plantar fascitis at one period of time during disease course).

Conclusions: Patients with enthesitis need regular follow-ups even if attained clinically important improvement, ongoing disease activity can cause increased damage accrual.


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Tofacitinib treatment in spondyloarthritis

Danveer Bhadu, Rudra P Goswami, Uma Kumar; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Introduction: Spondyloarthritis (SpA) is a group of diseases which involve spine and peripheral joints. Recently tofacitinib has been approved for the treatment of SpA by FDA. Though, safety and efficacy data for tofacitinib in SpA are very limited.

Objective: Aim of this study was to see the treatment response and safety of tofacitinib in SpA patients.

Methodology: SpA patients who fulfilled the inclusion and exclusion criteria were enrolled in this study after a written consent. All the clinical details [Table 1] were collected including disease activity before and after 2 months of tofacitinib therapy. Side effects of tofacitinib like drug intolerance, thrombotic event, infection etc were also monitored actively. Inclusion criteria: 1. SpA patients diagnosed as per ASAS classification criteria. 2. Juvenile Idiopathic Arthritis-Enthesitis Related (JIA-ERA) diagnosed as per ILAR classification criteria (age >18 years). 3. Patients with high or very disease activity (calculated as per ASDAS-ESR or CRP. Exclusion criteria: 1. Active or chronic infection 2. Past history of thrombotic events (DVT or thrombotic stroke or CAD).
Table 1: Clinical details of study population

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Results: Total 35 patients (M:F=33:2) were recruited in this study. Mean age of the study population was 31 years. In this study 27 adult SpA (77%), 6 JIA-ERA (17%) and 2 reactive arthritis (6%) patients were enrolled. Axial symptoms, peripheral joint, uveitis and HLA-B27 positivity was reported in 100%, 51%, 8.5% and 94% respectively. Disease activity [Table 2] calculated as per ASDAS-ESR or CRP was reported very high, high, moderate and inactive disease in 37%, 67%, 0% & 0% and 3%, 17%, 40% & 40% pre and post treatment respectively. Treatment response reported as major improvement, clinically significant improvement and no improvement in 37%, 49% and 14% of the patients respectively. Only one patient (3%) had reactivation of herpes zoster.
Figure 1: Effect of treatment on sleep improvement in psoriatic arthritis subject receiving standard treatment

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Table 2: Treatment response after 2 months of tofacitinib therapy

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Conclusion: Tofacitinib is effective and safe drug for the treatment of SpA patients.


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Undiagnosed depression in psoriatic arthritis

Charitha Sree, Nikhil, Roopa, Vijay K R Rao; Department of Rheumatology and Physiotherapy, Divisha Arthritis and Medical Center, Bangaluru, Karnataka, India

Background: Prevalence of depression in Psoriatic Arthritis (PsA) is not well known.

Objectives: The aim of this study was to evaluate the prevalence of undiagnosed depression in psoriatic arthritis (PsA) patients in a rheumatology clinic.

Methods: We performed retrospective questionnaire-based study using Hospital Anxiety and Depression Scale (HADS) as assessment tool.

Results: We had 110 patients with average age group of 47.3 years, majority females 60.9%. Obesity was seen in 54.5%. 6.3% had family history of psoriasis, 55.4% had cutaneous psoriasis (PsO), 75.45% had psoriatic arthritis (PsA) and 17.2% had nail psoriasis with overlap features in many. Disease duration of less than 2 years was seen in 66.36%. Mean ESR was normal in 64.5% and elevated in 35.45%. Mean CRP was normal in 45% and elevated in 55.45%. CSDMARDS were used in 86.3%, AntiTNF in 87.2%, NSAIDS in 63.6% and Steroids in 10.9% at any time in their disease. 63.6% of our patients followed diet control while 60% performed regular physical exercises. Using HADS as assessment tool to diagnose depression, there was a prevalence of 20% with undiagnosed depression. Among patients with depression 59% had PsO, 63.3% had PsA and 13.6 % had nail psoriasis. Sleep pattern before starting treatment was disturbed in 77.27% and improvement in sleep was observed in 90.9% after PSA treatment [Figure 1]. With Fischer's exact test, we observed statistically significant (p<0.0001) reduction in sleep disturbance among PsA patients on receiving standard treatment. There was no statistically significant difference in incidence of depression with physical exercises (p=0.1557), with diet control (p=0.0885) or combination of physical exercises and diet control compared to no physical activity (p=0.3330) and /or no diet control.

Conclusion: This study provides insight into undiagnosed depression and sleep disturbance in PsO and PsA patients.


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Faecal calprotectin in patients of ankylosing spondylitis with asymptomatic bowel disease and its relation to disease activity

Bhavya Chintala, Lalit Duggal1, Ved chaturvedi2, Neeraj Jain3, Mayank Gupta4; Department of Rheumatology and Clinical Immunology, Sir Gangaram Hospital, New Delhi, India

Background: Approximately 30%-60% patients with spondyloarthropathies have occult intestinal inflammation. Fecal proteins, such as calprotectin are indirect markers of neutrophil migration in the gut wall, and seem to be ideal for monitoring intestinal inflammation. By measure of fecal calprotectin level, we can detect subclinical gut inflammation early during disease course and mange appropriately. There is paucity in Indian studies regarding correlation of fecal calprotectin with spondyloarthritis disease activity in patients with asymptomatic bowel disease.
Table 1: Demographic characteristics of study population

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Objectives: Primary Objective was to study the prevalence of elevated fecal calprotectin level in patients of Ankylosing Spondylitis with asymptomatic bowel disease. The secondary Objective was to correlate elevated fecal calprotectin level with Ankylosing Spondylitis disease activity (measured by ASDAS CRP) and colonoscopic findings.

Methods: This study was conducted in 50 patients with spondyloarthritis in a tertiary care centre. All patients in the out -patient and in -patient departments of Rheumatology and Clinical immunology fulfilling ASAS classification criteria for Ankylosing Spondylitis aged more than 18years were included in the study. Patients with gastrointestinal symptoms suggestive of IBD like loose stools, blood and mucus in stool etc were excluded from the study. Correlation analysis was done by spearmann correlation and Fischer Extract test.

Result: The mean Age was 35.76 ± 10.97 years. Males:Females were 4:1.96% of the patients has inflammatory back pain and 70% of patients has peripheral pain. The mean duration of Symptoms (Years) was 4.61 ± 3.07. Uveitis is present in 16% of patients.84.0% of the participants had HLA-B27 Positive. 96.0% of the participants had B/L Sacrolitis on MRI. The mean CRP (mg/L) was 21.44 ± 22.35. The mean ASDAS CRP was 3.35 ± 0.92. 10.0% of the participants had Low Activity,46.0% had high disease activity and 44.0% had Very High Activity. The mean Fecal Calprotectin (μg/g) was 78.16 ± 121.70 with 51.0% of the participants had positive Fecal Calprotectin. Of these only 6 patients underwent colonoscopy and colonoscopic ulcers were present in 4 patients. There was a correlation between fecal calprotectin and ASDAS CRP (P value 0.045).

Conclusion: Fecal calprotectin was significantly associated with spondyloarthritis disease activity measured by ASDAS CRP. Fecal calprotectin can be used as a screening tool in patients with spondyloarthritis particularly with high disease activity to detect subclinical gut inflammation which aids in early diagnosis and management.


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Undiagnosed anxiety in psoriatic arthritis

Charitha Sree, Nikhil, Roopa, Vijay K R Rao; Department of Rheumatology and Physiotherapy, Divisha Arthritis and Medical Center, Bangaluru, Karanataka, India

Background: Prevalence of anxiety in Psoriatic Arthritis (PsA) is not well known.

Objectives: The aim of this study was to evaluate the prevalence of undiagnosed anxiety in Psoriatic Arthritis (PsA) patients in a rheumatology clinic.

Methods: We performed retrospective questionnaire-based study using Hospital Anxiety and Depression Scale (HADS) as assessment tool.

Results: We had 110 patients [Table 1] with average age group of 47.3 years, majority females 60.9%. Obesity was seen in 54.5%. 6.3% had family history of psoriasis, 55.4% had cutaneous psoriasis (PsO), 75.45% had Psoriatic Arthritis (PsA) and 17.2% had nail psoriasis with overlap features in many. Disease duration of less than 2 years was seen in 66.36%. Mean ESR was normal in 64.5% and elevated in 35.45%. Mean CRP was normal in 45% and elevated in 55.45%. CSDMARDS were used in 86.3%, AntiTNF in 87.2%, NSAIDS in 63.6% and Steroids in 10.9% at any time in their disease. 63.6% of our patients followed diet control while 60% performed regular physical exercises. Using HADS to diagnose anxiety, there was a prevalence of 27.2% with undiagnosed anxiety. Among patients with anxiety 60% had PsO, 63.3% had PsA and 13.3% had nail psoriasis. Sleep pattern before starting treatment was disturbed in 66.6% and improvement in sleep was observed in 93.3% after PsA treatment. With Fischer's exact test, we observed statistically significant (p<0.0001) reduction in sleep disturbance among PsA patients on receiving standard treatment [Figure 1]. There was no statistically significant difference in incidences of anxiety with physical activity (p=0.2790), diet control (p=0.1875) or combination of physical activity and diet control (p=0.1253) compared to no physical activity and /or no diet control.
Figure 1: Effect of treatment on sleep improvement in psoriatic arthritis subject receiving standard treatment

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Conclusion: This study provides insight into undiagnosed anxiety and sleep patterns in PsO and PsA patients.


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Tofacitinib is an effective and well tolerated drug in psoraitc arthritis patients – Real life experience

V N Nagaprabu, P Velammal1, A Gayathri, R Saranya, K Aswathi; Sakthi Rheumatology Centre Pvt Ltd, 1PSG Institute of Medical Sciences and Research Centre, Coimbatore, Tamil Nadu, India

Background: Tofacitinib a novel Janus Kinase Inhibitor (JAK) has been approved for the management of Psoriatic Arthritis (PsA), and has been shown to be effective. But the adverse events associated with JAK Inhibitors is always a concern and we are yet to gather some real life experience.

Objectives: To analyze the efficacy and safety of PsA patients treated with tofacitinib.

Methods: Case records of PsA patients who were treated with tofacitinib from January 2021 To June 2022 with minimum of 3 months follow-up were retrieved and their demographics, adverse events, details of investigations available were noted.

Results: There were a total of 52 PsA patients who were prescribed tofacitinib during this period out of which 40 patients fulfilled the inclusion criteria. Of the 40 patients there were 23 females and 17 males, with mean age of 45.5 (12.5) years. The mean disease duration was 5.29 (5.27) years. The mean duration of follow-up after tofacitinib intake was 8.65(3.66) months. 14 out of 40 discontinued the drug, out of which 11 were as they felt better and 3 in view of the persistent pain. One patient had Herpes and 4 had GI adverse events. But all of them responded well on re-challenging with the drug. 25 (71%) of 40 gained weight with mean gain In weight Of 3.316 (2.07) kilograms, while 8 had weight loss and 2 had no change in weight. Records were Hecords Were Not Available For 5. In the lab parameters there were significant reduction in CRP levels but others were not statistically significant [Table 1].

Conclusions: Tofacitinib in PSA patients is well tolerated and leads to significant reduction in CRP but also leads to weight gain (non-significant) which should be kept in mind.


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Great minds think alike, or do they?

S Sri Lakshmi, Deepti Agarwal, Sandeep Kansurkar, Kavita Krishna; Department of General Medicine and Rheumatology, Bharati Vidyapeeth Medical College and Hospital, Pune, Maharashtra, India

Introducion: Monitoring disease activity in spondyloarthropathies (SpA) is challenging. There is no single gold standard for measurement. We had surveyed the general thoughts and practices of disease monitoring in AS among doctors.

Objectives: To assess the current thoughts and practices of disease monitoring of AS among Doctors.

Methods: It is a prospective study conducted among Rheumatologists, orthopedicians and physicians. Their current thoughts and practices on disease monitoring among patients with AS was recorded. We used Google forms to record the response.

Results: Among the 40 participants, 74.1 % were rheumatologist, 18.5% were physicians and 24.4% were Orthopedicians. 68.4% responded that 20 to 30 years was the most common age group in patients with AS who presented to their clinics and 92.1 % had low back pain as their presenting complaint. Most of the participants said that the clinical and CRP were most likely used parameter to monitor disease activity. X Ray and MRI were less likely used. Among the clinical parameters BASDAI and ASDAS CRP was most likely used to tool [Figure 1]. Among imaging choices, 52 % preferred MRI to follow up disease activity and drug response, whereas 50 % preferred X-Ray [Figure 2]. Around 50 % wanted to add biologics even when was patient was in clinical remission but showed radiological progression.

Discussion: Disease monitoring in AS is very tricky as laboratory parameters usually do not correlate well with disease activity. And imaging modality like MRI is expensive and cumbersome for follow up of patients. Still clinical parameters are the choice among doctors to monitor the disease activity [Table 1].
Table 1: Demographic characteristics of study population

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Table 1: Variables

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Conclusion: Conclusive recommendations for monitoring patients with AS in clinical practice are lacking owing to the heterogenous presentation of the disease.


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Tofacitinib for the treatment of ankylosing spondylitis: Retrospective analysis

Divya Lala, Amruta Sanap1, Kunal Patil2, Yojana Gokhle3; Department of Rheumatology, Reliance Hospital/Fortis Hospital, 1Department of Rheumatology, Lokmanya Tilak Municipal Medical College and Sion Hospital, Mumbai, 2Sanjivani Rheumatology Clinic, Kolhapur, Maharashtra, India, 3Department of Rheumatology

Background: Tofacitinib is an oral Janus kinase inhibitor. The US FDA approved the use of tofacitinib in ankylosing spondylitis in December 2021. This study aims to analyse use of tofacitinib in adult patients with ankylosing spondylitis who do not respond to NSAIDs.

Objectives: Assess the efficacy of tofacitinib in adult patients with active ankylosing spondylitis (AS).

Methods: This is a retrospective analysis of patients with ankylosing spondylitis with inadequate response or intolerance to ≥2 non-steroidal anti-inflammatory drugs and conventional synthetic DMARD. Patients were receiving tofacitinib 5mg two times per day for minimum 12 weeks. The primary and key secondary endpoints were Assessment of SpondyloArthritis international Society ≥20% improvement (ASAS20) and ≥40% improvement (ASAS40) responses, respectively, at week 12.
Figure 1: Instruments you like use regulary in your practice to monitor the disease activity

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Figure 2: Choice of imaging for follow up of patients with AS

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Results: 23 patients were treated with tofacitinib, out of which 18 patients were males. The mean age of patients was 28.2. At week 12, the ASAS20 response rate was seen in 78.26 (18/23) patients and the ASAS40 response rate was seen in 69.56% (16/23). Four patients who had responded to biologics prior have shown a response to tofacitinib. There were no deaths, or major adverse cardiovascular events, thromboembolic events, or opportunistic infections.

Conclusions: tofacitinib can be very effective in patients with ankylosing spondylitis who do not show a response to NSAIDs.


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Gender-specific differences in clinical domains of patients with psoriatic disease at presentation to a tertiary care centre

K Illiasul Ibad, Ruchika Goel, John Mathew, Ashish J Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Prevalence of psoriatic arthritis (PsA) is equal in men and women. Sex-specific roles in driving mechanisms of PsA lead to differences in clinical manifestations of disease.

Objectives: We aimed to explore key differences in demographics, domain-specific clinical characteristics and comorbidities of men and women with PsA.

Methods: Patients over 18 years of age, diagnosed as PsA by the rheumatologist, attending the outpatient rheumatology clinics of a tertiary care hospital between January 2017 and June 2022 were included in this retrospective, cross-sectional study. Demographics, clinical features of PsA domains – nail, skin, peripheral joints, axial disease, enthesitis and dactylitis, and comorbidities of patients at their first visit to the clinics were retrieved from the electronic medical records. Gender-stratified differences among these clinical parameters were evaluated by Mann-Whitney U test and Pearson chi-square test or Fisher's exact test for continuous and categorical variables, respectively.

Results: A total of 300 patients, 180 (60%) men, with mean age ± standard deviation of 45.4± 13.12 were included. Details of demographics, clinical features and comorbidities are depicted in [Table 1]. Majority of the patients (men 85%, women 81.7%) had skin involvement at the onset of disease. Skin disease was the most common clinical feature at presentation, followed by peripheral arthritis in both genders. Axial disease was observed in 4.7% of patients. Women had significantly more polyarticular disease compared to men (40% vs 28.9%; p=0.046). Men had significantly more enthesitis and dactylitis compared to women (41.1% vs 27.5%; p=0.016 and 28.3% vs 15%; p=0.007, respectively). Diabetes and hypertension were the most common comorbidities in men and women, respectively. Men had significantly higher levels of inflammatory marker compared to women.

Conclusion: Active skin disease, enthesitis and dactylitis are more common in men and polyarthritis is more common in women with PsA in this cohort.


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Approximating ASDAS cut-off values with linear scales

Rudra Prosad Goswami, Shyamashis Das1, Moumita Chatterjee2; Department of Rheumatology, AIIMS, New Delhi, 1Department of Rheumatology, Institute of Neurosciences, 2Department of Mathematics and Statistics, Aliah University, Kolkata, West Bengal, India

Background: Measurement of disease activity among patients with spondyloarthritis (SpA) centres around the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the Ankylosing Spondylitis Disease Activity Score (ASDAS). ASDAS is a composite measure and needs a calculator, but has the advantage of cut-off values (1.3, 2.1 and 3.5) indicting higher grades of disease activity.

Objectives: We assessed various linear combinations of BASDAI, patient global assessment (PGA) and C-reactive protein (CRP, mg/L) to determine cut-off scores corresponding to the ASDAS (calculated with CRP) cut-off scores for the four disease activity states.

Methods: We collected data from our cohort of axial SpA patients on BASDAI, ASDAS, PGA and CRP, all taken in the same visit. Ordinal regression was run with ASDAS cut-off scores as dependent variable and different combinations of BASDAI, PGA and CRP as independent variables. For each regression cut-off scores were obtained according to previously described methods.1 Performance of the different scores were compared using the λp and τp.2 The degree of agreement between predictive scores and ASDAS cut-off values were assessed using weighted kappa.

Results: We included 285 patients of which 41 (14.4%), 96 (33.7%), 125 (43.9) and 23 (8.1%) patients had inactive disease, moderate disease activity, high disease activity and very high disease activity, respectively, according to ASDAS. Characteristics of the different linear combinations is given in [Table 1]. Maximum error reduction was seen with BASDAI only and adding any of PGA or CRP to BASDAI actually worsened prediction. Kappa statistic was also highest for BASDAI alone (0.51, p<0.001). The optimal cut-off scores of BASDAI obtained were 1.25, 2.5 and 6.
Figure 1: Decline of ASDAS-CRP at 1 month, 3 months and 6 months of treatment with tofacitinib

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Figure 2: Decline in BASDAI at 1 month, 3 months and 6 months of treatment with tofacitinib

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Table 1: Demographic, clinical features, comorbidities and laboratory marker in men and women with psoriatic arthritis

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Table 1: Performance statistics of the different linear combinations for correctly predicting the ankylosing spondylitis disease activity score cut -off scores

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Conclusions: The BASDAI values 1.25, 2.5 and 6 corresponded to the ASDAS values 1.3, 2.1 and 3.5, respectively. Addition of PGA or CRP did not improve performance of classification. These data-driven cut-off values could be useful in clinical studies and real-world practice.


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Evaluation of efficacy of tofacitinib in patients with refractory reactive arthritis: An observational study

M V Prakashini, Prasanta Padhan, Debashis Maikap; Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: Reactive arthritis (ReA) can be self-limited or chronic and develops after certain infections of the gastrointestinal or genitourinary tracts. There is a complex interplay of plenty of immune cells and cytokines, involving JAK pathway in ReA. Hence, we hypothesized that tofacitinib, a JAK-1/3 inhibitor already used in psoriatic arthritis would be effective in refractory ReA.

Objectives: Evaluation of efficacy and safety of Tofacitinib in patients with refractory ReA.

Materials and Methods: Fifteen patients attending the outpatient department of KIMS Bhubaneswar during the study period with refractory ReA were recruited on meeting the eligibility criteria defined by Braun et al (2000). Patients unresponsive to two different NSAIDs over four weeks along with intra-articular glucocorticoid, were defined as refractory ReA. They received 5mg tofacitinib twice daily. Outcome measures of disease activity and therapeutic efficacy were obtained at baseline, 1, 3 and 6 months. They included dactylitis, tender joint count, swollen joint count, BASDAI and ASDAS-CRP. Complete blood counts, liver and kidney function tests were monitored to assess adverse effects at baseline and follow up. All participants were followed for 6 months.

Results: Among fifteen patients with refractory ReA (Male:Female11:4, mean age of 37.6±15.76 years), twelve had HLA-B27 gene expression, 11 had dactylitis and 8 had axial involvement at presentation. Median CRP at baseline was 61 mg/L (IQR: 52-100). All patients had high disease activity at baseline (BASDAI: 5.67±0.92 and ASDAS-CRP: 4.6±0.7). On instituting treatment with tofacitinib, there was a gradual decline in ASDAS-CRP to 3.34±0.95, 2.71±1.4 and 1.1±0.14 at 1 month, 3 months and 6 months, respectively [Figure 1]. BASDAI showed a decline to 4.24±0.78, 3.31±0.99 and 1.72±0.67 at 1 month, 3 months and 6 months of follow-up, respectively [Figure 2]. Tofacitinib had a statistically significant effect on decline in disease activity and outcome measures – BASDAI and ASDAS-CRP at baseline and 6 months of treatment with p<0.001. None had relapse on follow-up. No adverse effects were recorded.

Conclusions: The clinical response of tofacitinib in refractory ReA was prompt without any adverse effects. Tofacitinib may be a good alternative to biologic therapy for treatment of refractory ReA especially in developing nations.


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Clinical outcomes of patients with psoriatic arthritis receiving biological DMARDS or tsDMARDS – A retrospective single centre study from South India: A real world experience

Donthula Grishma, S Rajeswari, C Balaji, C Saranya, Rashi Maheshwari, Joseph Antony; Department of Clinical Immunology and Rheumatology, Sri Ramachandra Medical College, Chennai, Tamil Nadu, India

Background: Psoriatic Arthritis (PsA) is a chronic inflammatory disease with multiple domains and available agents have different levels of effectiveness on each domain.

Objectives: Due to limited clinical data of these agents on Indian PsA patients, this study was intended to analyse their treatment outcomes.

Methods: Data was extracted from our single-centre 3-year Adult PsA cohort (>18 years; N=79). Patients treated with either Adalimumab (A)/Secukinumab (S)/Tofacitinib (T) were included (All patients also received 10mg Methotrexate). Primary objective was the proportion of patients achieving Major-Clinical-improvement (MjCR:ACR50 and ASAS40) at week-24. Change(s) in disease activity scores; mean response time (Tr), 6-month cost-effective analysis with incremental CE ratios (ICER) and drug survival were calculated. Logistic regression, AUROC and Kaplan-meier curves were computed where necessary.

Results: The mean age was 42.6 years (males: 62%) and 88% had peripheral arthritis [Figure 1] and [Figure 2]. ACR50-24W and ASAS40-24W were seen in 48%/51%, 41%/59%, 40%/39% of patients with A,S,T respectively [Figure 2]. At 24W mean change in PASDAS and PASI among the groups was similar; however, reduction in BASDAI score was higher with Secukinumab whereas proportion of patients free from Enthesitis-Dactylitis was higher with Adalimumab. Tr is similar across all 3 groups for ACR50 but to ASAS40, which was significantly higher with Tofacitinib [Figure 1] and [Figure 2]. For 6-month treatment cost, ICER (cost per unit incremental ACR [but not ASAS] response) of tofacitinib was significantly lower. Kaplan-meier-curves shows highest drug survival with Secukinumab. CRP (AUROC: 0.641; cut-off: 10.8, 71% accuracy]) and large joint involvement were the only predictors of MjCR and remission.
Figure 1: % of sudy population showing ACR50 and ASAS50 response with subgroup comparision

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Figure 2: Treatment response

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Table 1: Clinical profie of psoriatic arthritis patients at presentation

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Conclusions: Our South Indian PsA cohort study shows that Adalimumab/Secukinumab/ Tofacitinib are equally efficacious in improving arthritis. Secukinumab and Adalimumab may be preferable in patients with Axial and Enthesitis-Dactylitis symptoms respectively; but, adalimumab shows significantly lower time to axial clinical response. Tofacitinib was the most cost-effective option for arthritis and Secukinumab had highest drug survival. High-CRP (>10.8) and large joint involvement were poor prognostic markers.


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Use of RAPID3 in psoriatic arthritis

Charitha Sree, Nikhil, Roopa, Vijay K R Rao; Department of Rheumatology and Physiotherapy, Divisha Arthritis and Medical Center, Bengaluru, Karnataka, India

Background: Use of RAPID3 quality of life assessment tool in Psoriatic Arthritis (PsA) is not well known.

Objectives: The aim of this study was to evaluate the quality-of-life improvement in PsA patients in an out-patient rheumatology clinic.

Methods: We performed retrospective questionnaire-based study using RAPID 3 questionnaire as quality of life assessment assessment tool.

Results: We had 65 patients with average age group of 49.18 years, majority females 60%. Obesity was seen in 55.38%. 36% had family history of psoriasis, 66% had cutaneous psoriasis (PsO), 72.30% had PsA and 20% had nail psoriasis with overlap features in many. Disease duration of less than 2 years was seen in 40%. Mean ESR was normal in 69.23% and elevated in 30.76%. Mean CRP was normal in 42% and elevated in 58%. CSDMARDS were used in 87.69%, Anti-TNF in 89.23%, NSAIDS in 61.53% and Steroids in 6.15% at any time in their disease. Sleep pattern before starting treatment was disturbed in 52.30 % while improvement in sleep was observed in 96.92% after PsA treatment. Using Rapid3 score interpretation to categorise disease severity, before treatment, 15.3% were in near remission, 13.8% were in low severity, 36.92% were in moderate severity and 33.8% were in high severity categories. While after standard treatment, 40% were in near remission, 13.8% were in low severity, 30.7% were in moderate severity and 15.3% were in high severity categories. With Chi-square test we observed statistically high significant (P=0.0075) reduction in RAPID3 score with standard treatment [Figure 1] and [Figure 2]. With Pearson r test we observed statistically significant correlation between RAPID 3 score with mean CRP (P=0.0125) and mean ESR (P=0.0412) after treatment. With Pearson r test we observed a statistically significant (P=0.0317) reduction in sleep disturbance with standard treatment.
Figure 1: Correlation between CRP values versus RAPID3 score in PsA subjects

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Figure 2: Correlation between ESR values versus RAPID3 score in PsA subjects

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Conclusion: Disease severity, quality of life and sleep improvements in patients with PsA measured using RAPID3 tool were statistically significant after standard treatment. RAPID3 tool also corelated well with improvement in inflammatory markers after standard treatment in patients with PsA.


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JAK inhibitor – Tofacitinib - in the treatment of axial spondyloarthritis: A single center experience

Pravin Patil, Ridhima Jakhotia; Pune Rheumatology Center, Pune, Maharashtra, India

Background: Tofacitinib is an oral Janus kinase (JAK) inhibitor is approved in India for the treatment of rheumatoid arthritis, psoriatic arthritis and ulcerative colitis. In 2021, the US FDA approved tofacitinib for the management of Axial Spondyloarthritis (AxSpA). In India, the treatment paradigm for AxSpA primarily consisted of tumor necrosis factor inhibitors (TNFi) as advanced therapy. We report a series of adult patients with active AxSpA treated with tofacitinib.

Objective: To assess therapeutic effects of Tofacitinib in patients of AxSpA.

Methods: 150 patients included in the study fulfilled ASAS classification criteria for AxSpA. They received full dosage of tofacitinib for 3 consecutive months. The response to therapy was assessed by Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Bath Ankylosing Spondylitis Functional Index (BASFI), and inflammatory markers erythrocyte sedimentation rate (ESR) and c- reactive protein (CRP) at baseline and after 3 months therapy. Of the 150 patients, we report the results of 35 patients with a follow up of at least 3 months therapy with Tofacitinib.

Results: Out of 35 patients 28 were male (M: F – 4 :1) with a mean age of 33 years (18-47 years). The mean baseline BASDAI score was 5.57 (5.57 ± 1.54) and the BASFI score 4.33 (4.33 ± 2.13). The mean ESR and CRP at baseline were 42 (42 ± 23) and 31 (31 ± 18.5) respectively. When followed up, the mean BASDAI score improved to 1.92 (1.92 ±1.42) and BASFI 0.91 (0.91 ± 1.08). Significant improvement in ESR 15.7 (15.7 ± 9.8) and CRP 6.5 (6.5 ± 3.7) has been observed. There were no adverse events recorded during this short study.

Conclusion: Tofacitinib demonstrated rapid improvement in disease activity and functional index in patients of active AS and thus offers an alternative oral therapy for young adult patients with active AS.


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Study of inflammatory bowel disease associated arthritis

Mrunal Shelke, Ajit Ghadge, Aarti Zope, Amruta Bagul, Amruta Digole, Vipul Choudhary, Meghraj Ingle, Lalana Kalekar; Department of Internal Medicine and Rheumatology, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India

Aim: To study clinical profile & response to treatment of patients with Inflammatory Bowel Disease (IBD) associated arthritis.

Methods: It is a prospective study of 22 patients studied over 18 months in department of Medicine, in a tertiary care hospital, in western India. Patients aged >18 years diagnosed as IBD as per ECCO-ESGAR guidelines & having inflammatory arthritis (ASAS criteria) were included. Pregnant females were excluded. Baseline clinical parameters like type of arthritis, treatment history, disease activity (by CDAI/BASDAI) were noted. Baseline laboratory parameters like CRP, RA factor, HLA B27, Fecal calprotectin (FC), endoscopic & histopathological findings were noted. Patients were classified into Oligoarthritis /Polyarthritis/Axial arthritis, as per pattern of articular involvement. Periodic follow up of, improvement in CRP, CDAI and BASDAI score were noted every 3 monthly. Endoscopic resolutions were noted,at the end of 1 year. Chi- square test & paired t test were used. Level of significance was set at p<0.05.

Results: Out of 91 IBD patients screened, 22 (24.2%) had arthritis. Out of 22 patients, M: F=1:1, mean age was 35 years (30-40 years) [Table 1]. Axial arthropathy documented in 1, Oligoarthritis in 12 & Polyarthritis in 9, patients. At presentation, mean CRP was 33.4 mg/dL, mean FC was 341.8 mg/kg, mean CDAI was 15. Treatment used in our study was NSAIDS (100%), Mesalamine (63%), low dose steroids (22.7%), Methotrexate (27.7%), & Infliximab (9%). On follow up, decrease in, CRP 20/22 (91%), FC in 21/22 (95.4%) patients were noted. CDAI improvement in 20/21 (95.2%) (p <0.001), BASDAI improvement in 100% (p <0.001) were documented.
Table 1: Comparison of baseline and follow up characteristics of arthritis in inflammatory bowel disease

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Conclusion: In our study, IBD associated arthritis was noted in 24.2% patients. FC has 98% sensitivity & 91% specificity for diagnosis of IBD. Levels >120-200 mg/kg correlated with IBD diagnosis. In our study, IBD with Axial disease patients, refractory to conventional therapy responded to Infliximab.


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Disease course of axial spondyloarthritis during pregnancy

Sania Gore, Ritika Tambe, Asawari Raut, Pravin Patil; Poona College of Pharmacy, Bharati Vidyapeeth Deemed University, Pune Rheumatology Centre, Pune, Maharashtra, India

Background: Unlike other chronic inflammatory diseases, little is known about the disease course of axial spondyloarthritis (AxSpA) during pregnancy. AxSpA presents a unique scenario of difficulties due to anatomical as well as inflammatory effects on pregnancy.
Table 1: Disease course of AS

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Objectives: The aim of this study was to evaluate the course of disease during pregnancy.

Methods: An observational descriptive retrospective study was conducted to assess the disease course of AxSpA in pregnant females visiting a private rheumatology clinic. A self-administered questionnaire was used to collect data from 60 women who had been diagnosed with AxSpA before pregnancy.

Results: The average age at disease onset was 23.7 years. Just above 60% patients experienced ease of symptoms during pregnancy, 38% reported aggravation in symptoms and disease remained unaltered in only 2%. Half of the patients had to undergo caesarean section. Commonest symptoms during pregnancy were back pain and stiffness. Adverse pregnancy outcomes were reported by 16% patients. Postpartum flare of AxSpA was reported by 47% women. None of the patient had extra-articular manifestation during pregnancy or postpartum.

Conclusions: AxSpA has significant impact on pregnancy. Large number of patients reported worsening of symptoms during pregnancy and post-partum period.


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To study the T cell and cytokine profile in ankylosing spondylitis with effects of tacrolimus and tadalafil on the cytokines in-vitro

Pankti Mehta, Kritika Singh1, Mohit Kumar Rai1, Durga P Misra1, Vikas Agarwal1; Department of Clinical Immunology and Rheumatology, King George's Medical University, 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Most studies have shown mixed results of Interleukin (IL)-17 and IL-23 levels in patients with Ankylosing Spondylitis (AS). Targeting this pathway in humans has yielded benefits with reduced inflammation and radiologic progression. Controlling inflammation and fibrosis is the key to prevent osteoproliferation and tacrolimus and tadalafil have shown to inhibit these pathways.

Objectives: To study the:-T cell(Th17, PD1, CD25+CD4 T cells) and cytokine profile(IL-17, IL-23, TGF- β) in AS with influence of disease duration and activity-effect of tacrolimus and tadalafil in peripheral blood monocyte (PBMC) cultures to study their anti-inflammatory and anti-fibrotic potential.

Methods: Patients with AS with axial disease were recruited and their demographic, clinical profile, disease activity and radiologic indices were recorded. Peripheral blood was drawn and sera stored; PBMCs were isolated and cultured. Serum and PBMC culture supernatant were measured for above cytokines at baseline, after T-cell stimulation, and after treatment with tacrolimus (10 mM/ml), tadalafil (10 mM/ml) and both drugs combined by ELISA. The T cell profile was characterized by flow cytometry.

Results: Twenty-five patients [28(24-36);M:F, 7.3:1] were enrolled and the majority had moderate-high disease activity (ASDAS-CRP). The delay in diagnosis was significantly greater in patients with disease duration >36 months. Other parameters were similar across the groups [Table 1]. ASDAS-CRP showed a significant correlation with CD4Th17+ cells (r=0.7, p=0.07) and CD4 PD1/T reg (r=0.6, p=0.03). No significant correlation was observed between cytokines or T cell profiling with Modified Stokes Ankylosing Spondylitis Score. There was a significant decline in TGF-β levels in the culture supernatant with tacrolimus and tadalafil combined, however, no such difference was observed with IL-17 or IL-23 [Figure 1].
Figure 1: Cytokine levels in the peripheral blood monocyte culture supernatant at baseline, with anti- CD3, CD28 antibody, tacrolimus, tadalafil and tacrolimus + tadalafil

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Table 1: Comparison of characteristics between early and late disease

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Conclusions: Serum IL-17, IL-23 and TGF-β were not elevated in the peripheral blood without any influence of disease duration. A significant decline in TGF β was seen with combined tacrolimus and tadalafil. This needs further exploration in a larger sample size with subgroup characterization.


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Clinical profile of tofacitinib use in patients with ankylosing spondylitis: An observational study

Lekshmi Sreekumari Ramachandran, Hima Sreekumar, Vishad Viswanath, Anjana G Varier; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India

Background: Generic Tofacitinib is emerging as a cost-effective drug in the management of spondyloarthropathies. Safety profile is a major concern limiting its use. This study investigates whether early tapering can mitigate this risk and attain sustained remission.

Objective: To assess the clinical profile of generic tofacitinib use in adult patients with active ankylosing spondylitis (AS).

Methods: This retrospective observational study enrolled consecutive patients aged ≥18 years diagnosed with active AS, meeting the ASAS criteria. Patients received tofacitinib 5 and 10 mg per day. ASDAS CRP was calculated at initiation and after 6 months to monitor the response. The primary and secondary endpoints were assessment of ASDAS CRP at 6 months and early tapering of tofacitinib respectively. Assessment of safety profile was done regularly.

Results: 22 consecutive patients with ankylosing spondylitis on tofacitinib was followed up. The study showed a mild male preponderance (54%, 12 of 22). HLAB27 was positive in 77% (17 of 22) of the subjects. MRI evidence of sacroiliitis was found in 50% (11 of 22) and another 50% had features of joint hypermobility. Mean ASDAS CRP at the initiation of tofacitinib was 2.24. Tofacitinib dose of 10 mg per day was tolerated by 20 out of 22. After 6 months of treatment mean ASDAS CRP was 0.9 with attainment of remission in 84.2% (15 of 19). Average duration of tapering 4.5 months. Transaminitis occurred in 22% (5 of 22) and 2 patients (9%) had to be switched over to biologicals due to non-responsiveness. There were no deaths, malignancies, major adverse cardiovascular events, thromboembolic events, or opportunistic infections.

Conclusions: Tofacitinib can be considered as an effective treatment option in patients with AS who cannot afford biologicals. Early tapering of tofacitinib demonstrates no increased incidence of flares and use in select patients helps maintaining remission.
Table 1: Demography and clinical presentation of patients with axial spondyloarthritis at baseline visit

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Disease activity directed early tapering of TNF inhibitors in Indian patients with axial spondyloarthritis

Sindhuja Gopi, V Prabhu, Ashish J Mathew, John Mathew, Ruchika Goel; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Although recent studies have observed equivalence of disease directed tapering and continued standard dose of biologic DMARDS, current guidelines discourage their early tapering in axial spondyloarthritis (axSpA). In this retrospective study we ascertained the efficacy of early tapering of adalimumab biosimilar (ADA) for treatment of axSpA patients.

Methods: Patients diagnosed with axSpA who received early tapering as defined by reduction in frequency of ADA within 12 months of initiation during January 2021-2022 were included in this study. Data regarding demography, clinical features, disease activity, laboratory tests and medications were recorded from electornic medical records at baseline, and follow up visits either physically or via teleconsultation. Disease activity was defined according to ASDAS as per guidelines. The baseline parameters are presented as median (interquartile range, IQR) while the efficacy is described descriptively.

Results: Fifty-four among 110 patients received early tapering of TNFi [Table 1]. Tapering was initiated within 6 months (group A) and 1 year (group B) for 39 and 7 patients, respectively. ADA was administered 2 weekly until the first follow-up. Subsequently the dosing interval was increased by 1 week during every follow up visit for patients attaining inactive or low disease activity based on ASDAS. Median follow-up duration was 6 (IQR 3-8) and 4 (IQR 3-7) months for patients in group A and group B, respectively. Eight patients were excluded due to lack of follow-up. At the last follow-up, 36 (78.3%) patients were in either inactive or low disease activity, while 6 (13.0%) patients had active disease. Three and one patients discontinued the drug due to adverse events and financial constraints, respectively.



Conclusion: Disease activity directed early tapering of ADA was a practical option for maintaining low or no disease activity in patients with axSpA. Prospective longitudinal studies with longer follow up are required.


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To study the efficacy and NSAID sparing effect of conventional synthetic disease modifying anti rheumatic drugs in patients of spondyloarthritis

Juhi Dixit, Kriti Kishor, Kunal Chandwar, Dogga Prasanna Kumar, Digvijay Ekbote, Urmila Dhakad, Puneet Kumar; Department of Clinical Immunology and Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Use of csDMARDs in spondyloarthritis, although limited by sparse and inconclusive data regarding efficacy, might be the only affordable option in financially constrained settings.

Objective: To study the efficacy and NSAID sparing effect of conventional synthetic disease modifying anti-rheumatic drugs in patients of spondyloarthritis.

Methods: This was a single center prospective observational cohort study conducted over a period of 1 year in the Department of Clinical Immunology and Rheumatology, KGMU, Lucknow. Study included all newly diagnosed patients of spondyloarthritis who were conventional and biologic DMARD naïve and had active disease as defined by ASDAS-ESR score of ≥ 1.3. Patients were started upfront on sulfasalazine and methotrexate along with NSAIDs. Low dose oral and/or intra articular steroids were given wherever required. Clinical characteristics, laboratory parameters, radiological features, BADSAI, BASFI, BASMI, tender and swollen joint count, MASES, ASDAS-ESR and ASAS NSAID Index were recorded at baseline, 3 and 6 months follow up. Baseline parameters were compared to corresponding parameters and achievement of ASAS20 response was assessed at 3 and 6 months follow up.
Figure 1: NSAID sparing effect

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Results: Out of 150 patients enrolled, 78 completed 6 months follow up and were included in final analysis. Baseline characteristics are as shown in the Table. Mean ASDAS-ESR declined significantly to 2.14±1.03, 2.16±1.07, 2.11±0.96 for overall population, axial with peripheral arthritis subgroup and pure axial subgroup respectively at 6 months. ASAS20 response was achieved by 79.5%, 83.7%, 72.4% patients in overall population, axial with peripheral arthritis and pure axial subgroup respectively at 6 months. ASAS NSAID Index decreased significantly to 44.09±39.32, 49.52±40.03, 34.9±36.94 for overall population, axial with peripheral arthritis and pure axial subgroup respectively at 6 months. Other parameters also decreased significantly for overall population and both subgroups over 6 months.

Conclusion: CsDMARDS are efficacious and have NSAID sparing effect in patients of spondyloarthritis irrespective of presence of peripheral arthritis.


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A descriptive study on nature and treatment of psoriasis and development of metabolic diseases in patients with psoriatic arthritis

S Kadam, A Shaikh, P Jhadav, S Shinde, J Oak, A Erande, C Balakrishnan, K Bhojani, S Kalke, V Bagul, N Nolkha, P Mehta, R Samant; Department of Rheumatology, PD Hinduja National Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Background: Psoriatic arthritis (PsA) is a chronic, inflammatory arthritis associated with psoriasis (PsO). Estimates of PsA in patients with PsO vary from 20 to 30%. PsA has been shown to be strongly associated with obesity, diabetes mellitus.

Methods and Design: In this cross-sectional non-interventional study, patients with psoriatic arthritis were evaluated for psoriasis, past and current treatments, and cardio-metabolic risk factors. Data was collected over 12 months. Data recording was done in MS Excel by using Google Forms and descriptive statistics was done.

Results and Discussion: Examining the relation between nature, extent and treatment of baseline skin lesions and development of subsequent arthritis was one of the main aims of the study. Examining the prevalence of co-morbidities in PsA was another important aim. The predominant type of psoriasis was plaque (93%) followed by the rest. 76% of patients developed arthritis after skin involvement at an average of 6 yrs in our study. Very few patients received systemic therapy for skin psoriasis, majority received methotrexate (22.40%) followed by steroids and apremilast. The prevalence of type 2 DM was higher than the recently reported prevalence in India. On tallying hypertension and dyslipidemia, we noticed a similar prevalence of hypertension with as the national average, dyslipidemia had a marginally higher prevalence. It has been shown that PsA is associated with an increased risk of developing CVD, similar to our observations [Table 1] and [Table 2].
Table 1: Patient demographics and skin involvement and treatment in our patients

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Table 2: Prevalence of metabolic conditions

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Conclusion: We found the use of sustained systemic therapy for psoriasis (prior to onset of psoriatic arthritis) to be very low. We also found a much higher prevalence of obesity and diabetes in our patients with psoriatic arthritis compared to the general population, putting them at higher risk for cardio and cerebrovascular events. Early diagnosis and better treatment of psoriasis may hold the key to better control of psoriatic disease.


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Survey of disease awareness, management perceptions and care satisfaction in psoriatic arthritis patients from India: A multicentre study

Himanshu Pathak, Mohit Goyal, Vinod Ravindran, Bimlesh Pandey, Parthajit Das, Sham Santhanam, P Damodaran, Neeraj Jain, Saurabh Malaviya, Pravin Patil, Akshat Pandey, Prasandeep Rath, Anshul Goel, Arun Kumar Kedia, Amit Dua, Somya Jain, Ajaz Kariem Khan

Background: Psoriasis (Pso) is a common immune mediated dermatological disease which affects about 0.5% to 2.5% population of Indian population. About 8-10% of psoriasis patients get arthritis. There is evidence from worldwide studies that psoriatic arthritis (PsA) is associated with a high impact on the health-related quality of life and considerable cost, however, there is deficiency of data from Indian patients.

Objectives: The psoriatic arthritis patient survey is aimed to study Indian PsA patients' awareness and knowledge about the disease and treatment given and their satisfaction levels with the care which they are receiving. The results of survey will be increasing understanding of PsA patients' perception about their disease which will assist clinicians in giving holistic care.

Methodology: Our survey is a multicentred study conducted through members of Psoriatic Arthritis Special Interest Group (PASIG). PASIG is a group of Indian clinicians who are interested in research in psoriatic arthritis disease. As there were no previous similar studies from India, new structured validated survey templet was constructed for survey. Data collection was done for 12 weeks from seventeen centres across India. The validated survey proforma was translated in local languages (7 languages) for increasing patient participation. Written informed consent was taken from patients before participation and ethics committee approval was taken as per requirement. Data analysis was done in form of percentage, mean and median (IQR) for descriptive data and chi-square analysis done in nominal data for evaluation of significance.

Results: Total 274 PsA patients submitted the filled survey proforma in study period. In the respondents, 152 (55.47%) were males and 122 (44.52%) were females. The mean age of respondents 44.93±12.78 years. About 55% patients were of age group 18-45 years.

Conclusion: The survey results will be helpful in knowing the current status of disease perceptions in PsA patients across India. The information gained from survey can be used to increase awareness about PsA and formulating further research activities.


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Comparison of the efficacy and safety of biosimilar adalimumab injection with HUMIRA® in subjects with active ankylosing spondylitis

Amol Aiwale, Chandrashekara Srikantiah1, Jyoti Parida2, Archana Sonawale3, Vishnu Sharma4, Kaushik Basu5, John Mathew6, Chethana Dharmapalaiah7, Girish Bhatia8, Gaurav Seth9, Girish Kakade10, Neeraj Jain11, Reena Sharma12, Firdaus Fatima13, Rajeshwar Srivastav14, Romi Shah15, Bankim Desai16, Ajit Nalawade17, Vikram Haridas18, Uma Kumar19, R Naidu20, Roshan Pawar; 1ChanRe Rheumatology and Immunology Centre and Research, 7Medstar Speciality Hospital, Bengaluru, 18Sushruta Multispeciality Hospital and Research Centre Private Ltd, Hubli, Karnataka, 2Institute of Medical Science and SUM Hospital, Bhubaneshwar, Odisha, 3Seth G. S. Medical College and KEM Hospital, Mumbai, 8Medipoint Hospital, 10Sahyadri Superspeciality Hospital, 17Sancheti Institute for Orthopaedics and Rehabilitation, Pune, Maharashtra, 4Avron Hospitals Pvt. Ltd., 12Shalby Hospitals, Ahmedabad, 15Tristar Hospital, 16Nirmal Hospital, Surat, Gujarat, 5Medical College and Hospital, Kolkata, West Bengal, 6Christian Medical College, Vellore, Tamil Nadu, 9Akash Healthcare Super Specilaity Hopsital, 11Sir Ganga Ram Hospital, 19AIIMS, New Delhi, 13St Theresa's Multi Specialty Hospital, Hyderabad, Telangana, 14King George's Medical University, Lucknow, Uttar Pradesh, 20King George Hospital, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Aim: To compare the efficacy and safety of biosimilar adalimumab injection manufactured by Enzene Biosciences Ltd. with HUMIRA® in subjects with active ankylosing spondylitis (AS).

Objectives: Objective of the study to demonstrate the noninferiority of biosimilar adalimumab injection manufactured by Enzene Biosciences Ltd. with HUMIRA® wrt efficacy safety and immunogenicity in AS.

Methods: The prospective, multicenter, randomized, double-blind, phase III study involved 192 subjects with active AS recruited at 20 centers across India. The subjects who fulfilled the eligibility criteria were randomized in a ratio of 2:1 (i.e., 125 subjects in biosimilar adalimumab arm and 67 subjects in the innovator adalimumab arm). The selected subjects received both the investigational products at a dose of 40 mg subcutaneously (pre-filled syringe contained 40 mg adalimumab in 0.4 ml as the active ingredient) every other week for a total of 12 weeks. Efficacy assessment was done based on ASAS response criteria. Safety assessment was based on complete physical examination, adverse event (AE) monitoring, vital signs, ECG, anti-adalimumab antibody (ADA) assessment and laboratory tests.

Results: At 12 weeks, the ASAS 20/40/70 responses were achieved by 97.5%, 94.1%, and 68.9% patients who received biosimilar injection as compared to 98.4%, 96.7%, and 77.0% patients in HUMIRA® arm [Figure 1]. Safety assessment showed that 19 (15.2%) subjects reported 33 AEs in the biosimilar adalimumab arm and 8 (11.9%) subjects reported 11 AEs in HUMIRA® arm. ADA for positive and negative subjects was statistically non-significant (p-value: 0.3516) between the two arms.

Conclusion: The study has established the efficacy, safety and immunogenicity of biosimilar adalimumab when compared to HUMIRA® in patients with AS.


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To study the prevalence of depressive disorder in patients with osteoarthritis in tertiary care hospital in Central India

Apurva Khare, Sandeep Kumar Patel, Pritesh Goutam, Ashutosh Kumar, D P Singh, Lekhraj Garg; Departments of General Medicine and 1Psychiatry, L N Medical College, Bhopal, Madhya Pradesh, India

Background: Osteoarthritis (OA) is a chronic, degenerative, musculoskeletal disease characterized by degeneration of the articular cartilage and many of its surrounding tissues. It causes varying degrees of suffering amongst effected population. Depression is defined as serious mood disorder associated with persistent feelings of sadness, loss of interest and pleasure in daily activities. In India the prevalence of OA is in the range of 22% to 39%.

Objectives: To see the prevalence of depressive disorder in Osteoarthritis patients using standardized scale in Tertiary Care Hospital of Central India.

Methods: This is a Cross sectional Monocentric study done from September 2019 to October 2021, Patients more than 45 years who present with mechanical joint pain were taken, patients having family history of psychiatric illness, co-morbid psychiatric illness, having inflammatory arthritis, or having family history or active malignancy were excluded. Diagnosis of osteoarthritis was made clinically (Age >45 years, activity related joint pain, absent or minimal morning stiffness of <30 minutes), severity of pain was assessed using numeric pain rating scale (NPRS), Depressive disorder was assessed using HAM-D rating scale (17 item version) in questionnaire form.

Results: Out of 284 patients 95(33.5%) patients had depressive disorder in this study. 46 (16.20%) patients had mild depression, 33 (11.62%) had moderate depression and 16 (5.63%) patients had severe depression [Figure 1]. Data was analyzed using IBM SPSS ver. 25 software. Chi square test was used to find association of categorical variables [Table 1] and [Figure 1].
Figure 1: % of study population showing ACR50 and ASAS50 response with subgroup comparision

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Conclusion: Present study conclude that severity of depressive symptoms is directly related to pain severity, large proportion of patients suffering from pain have varying degree of depressive symptoms which was statistically significant, thus we conclude that assessment of mental health status of a patient with Osteoarthritis should form integral part of the disease management.


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Correlation between the clinical and radiological grading in knee osteoarthritis patients presenting to a tertiary cae centre in Kumaon region of Uttarakhand

Yashahvi Anmol Mishra, Deepali Joshi, Paramjeet Sing, Arun Joshi; Department of Medicine, Government Medical College, Haldwani, Uttarakhand, India

Introduction: Osteoarthritis (OA) is the most common joint disease of mankind and is also fast becoming an important cause of chronic disability in India. It is a slowly dynamic degenerative joint disease, characterized by pain and functional disability. The larger joints are commonly affected and specifically involvement of the hip and knee joint has a great health (care) and economic burden. Diagnosis of OA is usually based on symptoms (clinical OA) and is confirmed by radiography.

Objective: To study the correlation between the clinical and radiological grading in knee osteoarthritis patients presenting to a tertiary care centre in Kumaon region of Uttarakhand.
Figure 1: Proportion of patients with degrees of depression

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Figure 2: Correlation of presenting

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Methods: This cross-sectional study assessed the Clinicoradiological profile in Knee Osteoarthritis patients in Kumaon region of Uttarakhand in the Rheumatology Clinic, Dr. Susheela Tiwari Government Hospital, Haldwani, Nainital. The Radiographs was obtained of the affected Knee(s) which was then evaluated and hence graded via the K.L. Grading system. The patients were subjected to a questionnaire (KGMC Index, a modified WOMAC Index) that grades the patient's Pain, Stiffness and Functional Limitations at the affected knee(s) according to a 5 point Likert Scale. A correlation was hence determined between the Radiological and Clinical Grading.

Results: The mean KGMC SCORE was 37.48±8.61 (18-55). As per KL grading, majority had Grade 3 OA (37.2%) followed by Grade 4 (30.1%), Grade 2 (27.6%) and Grade 1 (5.1%). There was a significantly positive correlation of KGMC score with K.L. GRADING among the study population even when stratified into different age groups. Above the Healthy BMI Range of 18.5-24.9 kg/m2, an increasing BMI correlated both with an increasing K.L.Grade as well as with an increasing KGMC Score.
Table 1: Distribution of Hamilton depression rating scale score among osteoarthritis patients

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Conclusion: In case of knee osteoarthritis there is positive correlation between clinical and radiological grading in different age groups. The KGMC Index is an easy to use OPD based tool specifically formulated for the Indian population


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Association between trajectory of metabolic syndrome and knee pain over 11 years in middle-aged adults

Ambrish Singh1, Brooklyn Fraser1, Alison Venn1, Leigh Blizzard1, Changhai Ding1, 2, 3, Benny Antony1, 1Menzies Institute for Medical Research, University of Tasmania, Hobart, 3Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, Australia, 2Clinical Research Centre, Zhujiang Hospital, Southern Medical University, Guangzhou, China

Objective: Metabolic syndrome (MetS) is characterised by the clustering of central obesity with metabolic abnormalities. MetS has been suggested as having a role in osteoarthritis (OA) pathogenesis. To describe the association of MetS and trajectories of MetS over 10-13 years with knee symptoms in general population-based middle-aged adults.

Methods: Fasting blood biochemistry, waist circumference and blood pressure measures were collected during the Childhood Determinants of Adult Health (CDAH)-1 study (year: 2004-6; n=2447; mean age: 31.48±2.60) and at 10-13 year follow-up at CDAH-3 (year: 2014-2019; n=1549; mean age: 44±2.90). Participants were defined as having MetS as per International Diabetes Federation (IDF) definition. Participants were grouped in four MetS trajectories—'No MetS': no MetS at either life stage; 'Improved MetS': MetS only at young adulthood (CDAH-1); 'Incident MetS': MetS only at mid-adulthood (CDAH-3); and 'Persistent MetS': MetS at both life stages. Knee symptoms were assessed using the WOMAC scale at the CDAH-3. Univariable and multivariable (age, sex, and BMI adjusted) zero-inflated Poisson regression models were used for analysis.

Results: The prevalence of MetS increased from 8% at young adulthood (female: 52.06%) to 13% in mid-adulthood (female: 53.78%). Presence of MetS at mid-adulthood was associated with knee symptoms at mid-adulthood [ratio of means (RoM): 1.33; 95%CI: 1.27, 1.39]. Four MetS trajectories were identified—'No MetS' (85.01%); 'Improved MetS' (2.14%), 'Incident MetS' (8.81%), and 'Persistent MetS, (4.04%). Compared to 'No MetS' 'Persistent MetS' [RoM: 1.15; 95%CI: 1.06, 1.25], 'Incident MetS' [RoM: 1.56; 95%CI: 1.48, 1.65], and 'Improved MetS' [RoM: 1.22; 95%CI: 1.05, 1.41] was associated with higher knee symptoms. Notably, 'Incident MetS' was most strongly associated with knee symptoms [RoM: 1.56; 95%CI: 1.48, 1.65] and pain [RoM: 1.52; 95%CI: 1.37, 1.70] at follow-up.

Conclusion: In middle-aged adults, there was an independent positive association between MetS and knee symptoms. Relative to those without MetS at either life stage, the elevation in mean knee pain scores was more pronounced for those who developed MetS after young adulthood than those who had MetS in young adulthood.


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Prevalence of knee osteoarthritis in patients with type 2 diabetes attending diabetes clinic – Preliminary results

Siddharth Tiwari, Pooja Dhaon, Neeraj Tripathi, Kushal Singh1, R R Singh, Mukesh Shukla2; Departments of Medicine and 1Radiology, Hind Institute of Medical Sciences, Barabanki, Uttar Pradesh, 2Department of Community Medicine, VCSGGMSRI, Srinagar, Uttarakhand, India

Background: Knee osteoarthritis (knee OA) and type 2 diabetes (T2DM) contribute massively as a burden to the Indian society as both are highly prevalent. There are insufficient studies found with respect to their association despite having common risk factors.
Table 1: Characteristics of the participants at childhood determinants of adult health-1 and childhood determinants of adult health-3 follow-ups

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Objective: To study the prevalence of knee OA in patients with T2DM attending Diabetes clinic.

Methods: A cross sectional study, where in 150 (75 male and 75 female) patients of T2DM as per the ADA guidelines were enrolled. Demographic data and disease variables were recorded for all patients. All patients were assessed for knee OA as per clinico-radiological ACR criteria. Radiographic grading (Kellgren - Lawrence grading) was done taking right knee as index knee and functional status for knee OA was assessed using Western Ontario and Mcmaster Universities Index (WOMAC).

Results: Prevalence of knee OA was seen in 71 (47.3%) patients (34 males and 37 females). The mean age of patients was 57.7 + 10.2 years, mean BMI was 24.1 + 4.3 kg/m2, co-morbidity was seen in 59 (83.1 %) patients and the mean duration of T2 DM was 71.5 + 64.5 months. As per KL grading 61 (86%) patients had grade 2 OA and 10 (14%) had grade 3 OA. The mean knee pain on VAS (Visual Analogue Scale) was 41.76 + 21.5 mm and mean WOMAC score was 30.82 +17.9.

Conclusion: All the patients with T2DM must be assessed for the presence of Knee OA as a general routine care practice.


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A study on clinical profile of patients with hypermobile joints

Vishad Viswanath, Mary Catherine, Pooja Belani, Hima Sreekumar, Anjana G Varier, S R Lekshmi; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India

Background: Hypermobile joints are a common yet underrecognized cause for noninflammatory joint pains particularly in women.

Objectives: In this observational study, we describe the clinical profile of patients with Hypermobile joints.

Methods: 236 patients who presented to our clinic with joint pains with Beighton score more than 4 without concomitant inflammatory arthritis were selected for the study. Clinical data were collected and analysed.

Results: Mean age of patients was 41 years out of which 86% were females. Median duration of symptoms were 48 months. Median Beighton score was 7. Mean global VAS was 4. Criteria for Fibromyalgia was satisfied by 31% of patients. Other clinical features as described in [Table 1] were observed. Negative correlation was obtained between age and total Beighton score.
Table 2

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Conclusion: Hypermobile Joint Syndrome should be considered in patients presenting with nonspecific noninflammatory pains.


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Non surgical management of degenerative arthritis of temporomandibular joint: A 5 years prospective pilot study

Background: Degenerative Arthritis of TM Joint is the most common type of Arthritis and the most frequent cause of pain in that region. Limitation of jaw opening, chewing ability and joint noise. From Pre-anaesthetic check up clinic patients were referred to PMR department for this problem because they are not fit for G.A. due to difficulty in Endo-tracheal intubabation.

Objective: The purpose of this study was two-fold; to evaluate the outcome of Nonsurgical Management of Degenerative arthritis of TMJ and role & efficacy of conservative manage ment in TMJ Degenerative Arthritis providing consistent pain relief and functional jaw improvement.

Methods: The study was conducted in the department of PMR, S.N.P. Hospital, Kolkata.. Duration of study: since August 2004 to August 2009.24 patients (20 females and 4 males) involving 32 TMJTs (16 unilateral, 8 bilateral). Mean age group 37.5 yrs (maximum 45 yrs., minimum 30 yrs.); Maximum patients were from lower socio-economic group (60%), had addiction to tobacco / jarda /chutka chewing. Intervention: Patients were placed in 2 groups: Group A: 12 Patients (10 females, 2 males)-treatment started with Physical therapy, with thermal agents like cryo-therapy, heat therapy. Electro therapy (US Therapy, Iontophoresis) and TMJoint Exercises along with Intraoral splint. TMJ Exercises, behavioural modification to reduce aggravating factors, medications (like NSAID and muscle relaxants). Group B: In 12 patients (10 females, 2 males) Intra articular Injection of Triamcinolone (0.5 ml)+(0.5 ml of Lignocain 1%) were given in TM Joins under Fluroscopy guidance. Total 3 injections were given at 3 months interval. Information was gathered from patients' response questionnaires and clinico radi ographic Medical chart review. Change in pain intensity (Present treatment versus current) were measured by 2 Methods: 1) Pain experience (1 to 6) and Pain intensity (VAS scale 1 to 10), chewing ability, jaw opening and joint noise were evaluated (VAS 1 to 10).

Exclusion Criteria: 1. Infection, 2. Tumor of jaw, 3. Non cooperative patients, 4. Diabetes, 5. Other contra indications for modalities. During follow up period from Group A 2 patients did not turn up after 1 year and from Group B also 2 patients did not turn up after 9 months.

Results: A statistically significant (60%) improvement was noted after 6 months in Group B patients as compared to Group A (33%) improvement with p value <0.01.

Conclusion: Intra articular injection of steroid to TM joint responds promptly and is effective with acute symptoms that do not responds to other forms of medical management and provides significant pain relief and reduces TMJ dysfunctions in degenerative Arthritis of TM joints. But in long run physical therapy, thermal therapy (u/s therapy, iontophoresis) along with TMJ exercises like stretching exercise of TM joint with a Hallam's gag then with a dental gag, it is remarkable that this treatment should be effective within a couple of months and years, full painless movement is often restored without tendency to recurrence as compared to intra articula steroid injection and moreover long active steroid has got potential damaging effects. The results of this case series support further investigation of this form of Nonsurgical management in a rigorously controlled prospective fashion.
Table 1: Symptoms

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Association between osteoarthritis-related serum biochemical markers over 10–13 years and knee symptoms in middle-aged adults

Ambrish Singh1, Alison Venn1, Leigh Blizzard1, Brooklyn J Fraser1, Graeme Jones1, John Burgess1, 2, 3, Venkat Parameswaran1, 2, 3, Lyn March4,5, Flavia Cicuttini6, Changhai Ding1, 5, 7, Benny Antony1; 1Menzies Institute for Medical Research, University of Tasmania, 2Department of Endocrinology, Royal Hobart Hospital, Hobart, 5Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, 3Institute of Bone and Joint Research, Kolling Institute of Medical Research, University of Sydney, 4Department of Rheumatology, University of Sydney Royal North Shore Hospital, Sydney, Australia, 7Clinical Research Centre, Zhujiang Hospital, Southern Medical University, Guangzhou, China

Background: Serum levels of cartilage and joint-specific biochemical markers are associated with cartilage degradation, joint tissue degradation, and synovitis in patients with OA. However, few studies have evaluated the association of OA-related biomarkers with knee symptoms in the general population-based middle-aged adults.

Objectives: To describe the associations between OA-related biochemical markers and knee symptoms in middle-aged adults followed up over 10-13 years.

Methods: Blood samples were collected during the Childhood Determinants of Adult Health (CDAH)-1 study (year: 2004-06) and 10-13 year follow-up at CDAH-3. Serum samples from baseline (n=156) and follow-up (n=167) were analyzed for three OA-related biomarkers [cartilage oligomeric matrix protein (COMP), matrix metalloproteinase (MMP)-3, and hyaluronan (HA)] using non-isotopic enzyme-linked immunosorbent assay (ELISA). Knee symptoms were assessed using the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scale at follow-up. Univariable and multivariable (adjusted for age, sex, and body mass index (BMI)) zero-inflated Poisson (ZIP) regression models were used for analysis.

Results: In cross-sectional multivariable models, significant positive associations between COMP [ratio of means (RoM): 1.156; 95%CI: 0.989, 1.324], MMP-3 [RoM: 1.013; 95%CI: 1.001, 1.025], and HA [RoM: 1.008; 95%CI: 1.002, 1.015] with knee pain and WOMAC-total score were observed in middle-aged adults. Baseline serum MMP-3 [RoM: 1.013; 95%CI: 1.006, 1.020], sum of serum COMP [RoM: 1.022; 95%CI: 1.011, 1.033], and an increase in the serum HA [RoM: 1.014; 95%CI: 1.007, 1.020] were significantly positively associated with knee pain assessed after 10-13 years.

Conclusion: The cumulative COMP levels in young adulthood (baseline) and mid-adulthood (follow-up), MMP-3 levels in young adulthood (baseline), and change in HA levels over the follow-up period were positively associated with knee pain assessed after 10-13 years. These biochemical markers measured in middle-aged adults may be explored further as a predictor of future knee symptoms.


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A multicentre study of clinical profile of Juvenile localized scleroderma in Indian children (conducted by Pediatric Rheumatology Indian Collaborative Experience under the Aegis of Pediatric Rheumatology Society of India)

Mahesh Janarthanan, Archana Khan1, Anushka Prabhudesai1, Suma Balan2, Sekhar Easwar2, Anand Rao3, Manjari Agarwal4, Mahabaleshwar Mamadapur5, Sagar Bhattad6, Priyankar Pal7, R Subramanian8, Suparna Guha9, Vijay Viswanathan10, Jyothi Raghuram11, Girish Subramaniam12, Raju Khubchandani1; Sri Ramachandra Institute of Higher Education and Research, 5Madras Medical College, Chennai, Tamil Nadu, 1SRCC Children's Hospital, Mumbai, 10Jupiter Hospital, Thane, 12Children's Hospital, Nagpur, Maharashtra, 2Amrita Institute of Medical Sciences, Kochi, Kerala, 3Manipal Hospital, 6Aster CMI Hospital, 11Aster Women's and Children's Hospital, Bengaluru, 8JSS Medical College, 8JSS Medical College, Mysuru, Karnataka, 4Sir Ganga Ram Hospital, New Delhi, 7Institute of Child Health, 9Vivekananda Institute of Medical Sciences, Kolkata, West Bengal, India

Background: Localized scleroderma, a rare autoimmune disease characterized by skin thickening and excessive accumulation of collagen with fibrosis leading to atrophy of deeper subcutaneous tissues, muscles and bones. Sequelae include cosmetic problems, joint contractures, limb length discrepancy, facial atrophy and extra dermal complications such as ocular, musculoskeletal or neurological.

Objectives: To describe the clinical profile, subtypes and management of JLS in an Indian cohort.

Methods: Retrospective data of JLS patients was collected online from 13 centres in a standardised questionnaire. This included sex, age of onset, current age, time to diagnosis, family history of connective tissue disorders, type of scleroderma, parts of body involved, diagnostic method, tests for autoimmunity, extra- cutaneous manifestations, treatment, follow up and complications.

Results: Data from 114 children from 13 centres was received. The M: F ratio was 4:7. Disease onset occurred most commonly between 5 and 10 years of age (46.5%). For 30/114 children time to diagnosis was more than 2 years. There was no family history of connective tissue disorder in any of the patients. Linear scleroderma 44 (38.6), morphea (single small circumscribed lesion) 23 (20.2%), mixed (16.7%), Encoup de sabre11 (9.6%), Parry Romberg 10 (8.7%) were the commonest types. Face 38 (33.3%), abdomen 35 (30.7%), right leg 33 (28.9%) were the common body parts involved. A clinical diagnosis of scleroderma was done in 95 (83.3%). Tests for autoimmunity was done by 82 (71.9%) and ANA was positive in 53 (64.6%). Arthritis was the most common extra cutaneous manifestation. Methotrexate 100 (87.7%) was the most common DMARD used in treatment. Adherence to treatment was good in 92 (80.7%) and serial photos at every visit was the monitoring tool in 89 (78.1%) children. Liver function abnormalities were noted in 15 (13.2%). Surgical procedure was done in 8 (7%). Cosmetic complications were noted in 59 (51.8%). Of children who were followed up for at least a year, no further progression of lesions was noted in 51 (44.7%).

Conclusion: We present data of 114 children with localized scleroderma, the first of its kind from India.


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Prevalence and predictors of macrophage activation syndrome in Kawasaki disease: A single center observational study

Vanshika Kakkar, Anu Maheshwari, Sameer Gulati1, D R Kavya, Adiba shakeel, Ashok Kannepalli, Jefi Rajan, Meenakshi Aggarwal2, Srikant Basu, Deonath Mahto; Departments of Pediatrics and 2Microbiology, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, 1Department of Internal Medicine, Lady Hardinge Medical College and Associated Smt Sucheta Kriplani Hospital, New Delhi, India

Background: Beginning of the SARS-CoV-2 pandemic was marked by an increase in the incidence of Kawasaki Disease (KD) and its antecedent complications like Macrophage activation syndrome (MAS), overt myocarditis and Kawasaki Disease Shock Syndrome (KDSS).

Objectives: We aimed to study the prevalence and the early predictors of MAS in patients with KD.

Methods: All children in the age group of 1-18 years admitted in our tertiary care hospital during the period of March 2020 to October 2021 who were diagnosed with KD as per AHA 2017 and had a negative SARV-CoV-2 serology/ RTPCR were enrolled. All patients were investigated for MAS as per International Consensus criteria by Ravelli et al. A comparative analysis of clinical and laboratory parameters was done in KD patients with and without MAS. Logistic regression analysis was done for predicting the odds of MAS in KD and a classification tree was built to predict MAS in KD.

Results: A total of 42 patients were enrolled in the study out of which 14 (33.3 %) were diagnosed with MAS. The KD patients with and without MAS did not show any significant difference in the clinical phenotype as shown in [Table 1]. As per multivariate logistic regression analysis, children with underlying myocardial dysfunction, hypoalbuminemia of <2.8 gm/dl, duration of fever >6 days were strong predictors of MAS. The classification tree generated by modelling is shown in [Figure 1].

Conclusion: MAS remains an under-reported entity with grave outcomes in patients with KD due to a significant clinical overlap. There has been a surge in the prevalence of inflammatory disorders including KD and associated MAS reported during the SARS-COV2 pandemic. Early screening for MAS using easily available bedside parameters can help in early detection and timely institution of specific therapy and improve disease outcomes.


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Study of growth and development in patients with juvenile idiopathic arthritis – Enthesitis related arthritis variants

Sayan Mukherjee, Abilash V Krishnan, Nishant G kamble, P M Ankush, Mukesh K Maurya, Pankti Mehta, T G Sundaram, Puneet kumar, Urmila Dhakad; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Juvenile Idiopathic Arthritis (JIA) is an umbrella term for chronic arthritis with onset before 16 years of age. According to ILAR classification criteria it is subdivided in 6 category. Growth failure is a well observed phenomenon in JIA and more so in Systemic onset and polyarticular variants. Except from western literature, in Asian countries Enthesitis Related arthritis (ERA) is more prevalent. In our study, we have studied the growth and development of patients with Enthesitis Related arthritis variant.

Objectives: To assess the prevalence of growth failure and sexual maturity in patients with JIA-ERA and possible association with disease activity, damage and drug therapy.

Methods: We have enrolled all patients of JIA-ERA, visiting rheumatology outdoor patient service in KGMU, Lucknow, from July 2021 to Dec 2021. All the patients must qualify Revised ILAR classification criteria for ERA. Those who have concomitant developmental disorders, secondary amyloidosis and macrophage activation syndrome were excluded from the study. All patients were clinically examined and previous medical records were thoroughly checked. Anthropometric measurement and tanner staging was done at baseline. Growth velocity was observed at follow up visit. This is a Prospective observational study with follow up at 3 and 6 months.

Results: 32 patients of JIA-ERA were enrolled. Majority of them were boys with a male to female ratio of 4.3:1 and median age of 15.5 years. Median age of onset was 11.5 years with a mean disease duration of 41.25 months. 31.2% had family history of SpA in first degree relatives and 12.5 % had history of uveitis in parents. Only 2 patients (6.25%) had history of other autoimmune diseases in family. There was no developmental delays in children with JIA-ERA and 30 patients (93.75%) were vaccinated for their age. 75% patients had inflammatory back pain with significant early morning stiffness at baseline and 18% had purely axial involvement. 18.7% had active or past history of uveitis, 12% had dactylitis and 25% had enthesitis (predominantly Achilles enthesitis). 75% had achieved age appropriate linear growth with 18.75% were 2SD above their mid parental height. 10 patients (31.25%) had significant limb length discrepancies. 62.5% were underweight for their age with low BMI. 30% patients had delayed sexual maturity in terms of sparse axillary and pubic hair with low tanner stage for that age. In majority of cases growth velocity of 4cm/6month was observed which was quite similar to the pubertal growth spurt of normal reference value. No significant association of growth failure was observed with prior corticosteroid use, disease duration, disease activity (JSPADA and JADAS27) and damage (JADI) score.
Figure 1: Classification Tree predicting MAS in KD patients. Terminal nodes 3, 5, 6, 10, 11, 12 and 13 depict the proportions of patients with and without MAS. Terminal nodes 6 and 12 included only patients with MAS. The classification rules for these nodes may be utilized for predicting MAS in KD patients at bedside

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Table 1: Clinical profile and laboratory parameters of Kawasaki disease patients with and without macrophage activation syndrome

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Conclusions: Growth failure wasn't well observed in JIA-ERA variants. One third had delayed sexual maturity for their age. Growth velocity was similar to age matched healthy population. No significant association was found with prior corticosteroid use, disease duration, disease activity or damage score.


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IgA anti CD74 antibodies in the serum of enthesitis related arthritis category of Juvenile idiopathic arthritis

Anu Balakrishnan, Sanjukta Majumder, Shivika Guleria, Able Lawrence, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, SGPGIMS, Lucknow, Uttar Pradesh, India

Introduction: Enthesitis related arthritis (ERA) is the most common type of JIA in India. ERA has some similarities with the adult counterpart, Ankylosing Spondylitis (AS). MIF was found to have role in the pathogenesis of AS. Antibody to its receptor CD74 was also found in AS in multiple studies.

Methods: Patients with ERA fulfilling ILAR classification (n=89) is recruited and compared to AS patients (n=26), and healthy controls (n=17). Serum IgA anti CD74 antibodies was measured by ELISA using Aeskulisa SpA Detect Kits.

Results: Majority of the patients were male with M:F ratio of 77:12, and median age was 16.39 (7-25), and duration of disease of 56.88 (12-240) months. Majority of these patients had arthritis (70.78%), enthesitis (56.18%), uveitis (21.35%), sacroiliitis (14.61%) and, inflammatory back pain in 19.10%. HLA B27 was present in 85.39%. Only 3/89 (3.41%) patients of ERA had IgA antiCD74 antibody whereas it was not present in any patient with AS or healthy control.

Conclusion: IgA anti CD74 antibodies are rarely seen in ERA. This may be because of the difference in the genetic composition or because of the lesser prevalence of gut inflammation in spondylitis in the Asian population compared to the Europeans.


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Levels of oxidized-LDL in children with juvenile idiopathic arthritis compared to healthy control – A cross sectional observational study

Jefi Rajan, Deonath Mahto, Anu Maheshwari, Ritu Singh; Departments of Paediatrics – Paediatric Rheumatology and 1Biochemistry, Lady Hardinge Medical College, New Delhi, India

Background: Increased risk of atherosclerosis and cardiovascular disease in children with autoimmune disease is well known. Conventional lipid profile parameters do not adequately reflect the true atherosclerotic potential in every JIA patient. So, the purpose of our study was to look for a biomarker that accurately predicts the same and correlation of disease activity with Ox-LDL and thereby aiding in control of inflammation and atherosclerosis.

Objectives: To evaluate the levels of lipid profile and Oxidized-LDL in children with juvenile idiopathic arthritis and correlation of disease activity (JADAS 27) with Ox-LDL and lipid profile – thereby to assess the atherosclerotic risk potential in uncontrolled disease.

Methods: Seventy-two children of age group 6months -18yrs fulfilling ILAR criteria of JIA and seventy-two age and sex matched healthy controls attending OPD for minor illness were included in our Cross-sectional observational study and Lipid profile (Total cholesterol, HDL, Triglycerides, LDL) and Ox-LDL were assessed in both JIA population and healthy controls.

Results: On comparing the levels of Lipid profile and Ox-LDL between cases and controls- significant difference was seen in Total cholesterol (mg/dl) (P value <0.001), HDL (mg/dl) (P value <0.001), Triglycerides (mg/dl) (P value <0.001), Ox-LDL (ng/ml) (P value <0.001) but not in LDL (mg/dl) (P value = 0.20). When compared with disease activity (JADAS-27) lipid profile parameters showed no significant correlation, however Ox-LDL showed a significant correlation with r value = 0.455 and p value < 0.005.

Conclusion: Broadly atherogenic lipid profile pattern was seen in our JIA population and though there was no significant correlation between conventional lipid profile parameters, significant correlation of disease activity (JADAS 27) with Ox-LDL was seen. Thus, use of these strategy favouring early identification underlying inflammation and aggressive use of available effective anti-inflammatory medication in controlling the disease activity for better vascular health of these young population should be considered.


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Myositis-specific and myositis-associated autoantibodies in juvenile dermatomyositis

Mahabaleshwar Mamadapur, P S Arul RajaMurugan, S Ramesh, S Mythili, S Sreenath; Department of Clinical Immunology and Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Juvenile Dermatomyositis is the most common inflammatory myopathy in children. It is characterized by proximal muscle weakness with typical cutaneous manifestations and has a propensity for multisystemic involvement. The antibody profile has a significant correlation with disease manifestations and risk of complications. There are very few studies on the autoantibody profile in JDM.

Objectives: To determine the myositis autoantibody profile in JDM and correlate with clinical phenotype.

Methods: Children who were diagnosed with JDM (newly diagnosed during study period or old) were included in the study. 19 patients were included during the study period from Dec 2020 to May 2022. Autoantibodies were detected using Euroimmune kit. Autoantibodies studied were La,Ro52,OJ,EJ,PL-12,PL-7,SRP,JO-1,PM75,PM100,Ku,SAE1,NXP2,MDA5,TIFϒ,Mi-2a,Mi-2b.

Conclusion: In this small cohort of 20 patients,the overall positivity rate of 70% for autoantibodies is similar to other studies. Amit Rawat et al. did a similar study and the autoantibody positivity was 30%. They attributed this difference to ethnic variation. Further studies are needed in larger collaboration.


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Multitargeted therapy for refractory childhood SLE: Usage, safety and efficacy profile from North India

Manjari Agarwal, Devang Pandya, Sujata Sawhney; Department of Pediatric Rheumatology, Sir Ganga Ram Hospital, New Delhi, India

Background: Childhood lupus nephritis is difficult to manage and can be resistant to first line agents. Steroid usage is significant and leads to significant morbidity. Multitargeted therapy is used in adult patients successfully. Addition of tacrolimus to mycophenolate mofetil can be helpful and be used as steroid sparing combination for not only nephritis but other clinical manifestations of childhood SLE as well.

Objectives: To study the usage, safety and efficacy of a combination of mycophenolate mofetil and tacrolimus in childhood SLE.

Methods: Retrospective study. All children diagnosed with SLE who received a combination of mycophenolate mofetil and tacrolimus for more than 6 months were included. Children who received the combination for less than 6 months were excluded from the study.

Results: Combination of mycophenolate and tacrolimus was used in 16 children, 12 had nephritis and 4 children were given the combination due to persistent pyrexia with requirement of high doses of corticosteroids.4 boys and 12 girls formed the study cohort. Median duration of combination used is 23.5 months (IQR 7-48 months). Three children had rising creatinine, one had to interrupt the drug due to this. One child had loose stools and rising creatinine and a dose reduction of tacrolimus was required. Leucopenia, transaminitis were not seen with the combination usage. A trough tacrolimus level of 5-10 ng/ml was targeted for all. Median dose of tacrolimus was 0.06 mg/kg/day (IQR 0.02-0.13). There were no major adverse events on the use of this combination. Median creatinine level prior to combination use was 0.50 mg/dl (IQR 0.3-0.94) and after 6 months of use was 0.53 (IQR 0.35-1.49) [Table 1],[Table 2],[Table 3].
Figure 1: Lipid profile and Ox-LDL with various subtypes of JIA

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Figure 2: Scatterplot showing correlation between oxidized LDL with disease activity scores (JADAS 27) in children with JIA

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Table 1: Patients with positive autoantibodies (n=14)

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Table 2: Frequency of positive antibodies in our study

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Table 3: Clinical manifestations, lab investigations and treatment details of study patients

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Conclusions: Combination therapy of mycophenolate mofetil and tacrolimus was well tolerated by most children. It was useful in reducing the dose of steroids successfully and was helpful in attaining long term steroid free remission in children. In some children, an abrupt increase in creatinine was noted and the combination was discontinued.


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Mixed connective tissue disease in children – An Indian perspective

Suparna Guha, Deepti Suri1, Suma Balan2, Mahesh Janarthanan3, Manjari Agarwal4, Vijay Viswanathan5, Aman Gupta6, Rashna Dass Hazarika7, Sagar Bhattad8, Anjani Gummadi9, Murugan Sudhakar2, Samar Ranjan Pal, Jyothi Raghuram10, Jyothi Srikkanth11, Anand Prahalad Rao12, Amita Aggarwal13; Vivekananda Institute of Medical Sciences, Kolkata, West Bengal, 1Advanced Pediatrics Centre, PGIMER, Chandigarh, 2AIMS, 11Rheumatology and Clinical Immunology, AIMS, Kochi, Kerala, 3Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, 4Sir Gangaram Hospital, New Delhi, 5Jupiter Hospital, Thane, Maharashtra, 6Medens Hospital, Panchkula, Haryana, 7Rigpa Children Clinic and Nemcare Superspeciality Hospital, Guwahati, Assam, 8Aster CMI Hospital, 12Manipal Hospital, Bengaluru, Karnataka, 9Ankura Hospital For Women and Children, Hyderabad, Telangana, 13Department of Clinical Immunology and Rheumatology, SGPGI, Lucknow, Uttar Pradesh, India

Background: Mixed Connective Tissue Disease (MCTD) in children is a rare entity. There is a paucity of studies on Juvenile MCTD (jMCTD) worldwide especially from the South- East Asia. It is still not known how and to what extent pediatric onset disease differs from the adult onset variety. Though few studies have shown a milder course in children some on the contrary have observed a less favourable outcome compared to adults. In India there have been several studies on MCTD in adults but there is lack of data on jMCTD.

Methods: Major pediatric rheumatology centers across India were invited to participate in this retrospective, case record review-based study, to analyze the clinical features and outcome of children with MCTD. All patients with physician diagnosed MCTD less than 18 years were included in the study. Overlap syndromes and other connective tissue diseases were excluded from the study. Data collected included age of onset, duration of illness, initial presenting features, organ specific involvement, investigations performed in the form of routine blood test, serology, and necessary imaging like CXR,CT chest, ECHO, and pulmonary function test. Treatment provided and outcomes including years of follow up, complications and current status was recorded.

Results: Data for 31 patients (girls 27, boys 4) from 11 centers were included. Among these 31 cases, 17 fulfilled Kasukawa criteria, 10 Alarcon-Sergovia, 1 Sharp and 3 satisfied all the three criteria. The median age at presentation was 12 years (range 5-18 years) and median duration of symptoms was 24 months (range 2-96 months). The common features included arthritis (90%), Raynaud's phenomenon (70.96%) and puffy fingers (67.74%). At a median duration of follow up of 24 months (1-168 months), 45% went into complete remission. There were 2 deaths due to macrophage activation syndrome and overwhelming sepsis.

Conclusion: Though very rare, constellation of typical findings in a patient with constitutional symptoms like fever should alert the treating pediatrician to consider MCTD as one of the differential diagnoses. The initial clinical features of MCTD in our cohort was similar to other studies. The course of the disease was favourable with minimal organ involvement seen on follow-up. The clinical presentation of our pediatric cohort had many similarities with the adult Indian cohort, although systemic involvement (renal, CNS and gastrointestinal) was rare. The major limitation of our study was the retrospective nature, small patient numbers and very short period of follow-up. However, it does provide some insight into the milder clinical phenotype and favourable prognosis of the disease in children in the Indian sub-continent.


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Clinical presentation of rheumatic diseases in children – Single center experience from Western India

Meghana Nadagoudar, Sandeep Kansurkar, Jitendra Oswal, S Srilakshmi, Kavita Krishna, Deepti Agarwal; Bharati Vidyapeeth Medical College and Hospital, Pune, Maharashtra, India

Background: Rheumatic diseases in children have unique patterns of presentation which may be significantly different from their adult counterparts. Data for these presenting manifestations of pediatric rheumatologic diseases is scarce from western India.
Table 1: Highlights the usage and efficacy of the combination of mycophenolate and tacrolimus

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Objectives: This study is a brief analysis of data from our registry of rheumatic diseases in children.

Method: The registry contains clinical information of subjects who were below 18 yrs of age and diagnosed with rheumatic diseases. Frequency of common rheumatic complaints were retrieved. Other parameters like type of joints involved, systemic manifestations and ongoing dose of glucocorticoids were also recorded.

Results: Registry contained information of 186 subjects. 46% were female. Commonest complaint was joint pain and involvement of large joints was more common than small joints. Distribution of common rheumatological complaints is shown in [Figure 1]. Frequency of internal organ involvement is shown in [Figure 2]. Anemia was seen in half of the cases and gastrointestinal system was most common extra-articular organ involvement. Most common diagnosis was Oligoarticular juvenile idiopathic arthritis (OJIA). Of all cases, 56% were already receiving some form of glucocorticoid treatment. [Figure 3] compares final diagnoses recorded in this study. Pediatric Multisystem Inflammatory Syndrome was seen in 6% of subjects.

Conclusion: OJIA is commonest arthritis in children in western India as compared to Enthesitis-related arthritis reported in other parts of India. Pediatric Multisystem Inflammatory Syndrome is important new entrant in this category of patients.


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Kawasaki disease: Impressions from a newly established pediatric rheumatology cell in North-West India

Avinash Sharma, Sandesh Guleria; Dr Rajendra Prasad Government Medical College, Kangra, Himachal Pradesh, India

Background: Kawasaki disease is a common medium vessel vasculitis of childhood that can result in serious coronary artery complications in a significant proportion if not managed appropriately. We here present our experience with patients of Kawasaki Disease managed at Dr Rajendra Prasad Government Medical College, Tanda, Kangra, Himachal Pradesh, India.

Objective: To study the epidemiology of Kawasaki Disease (KD) over time among children in Dr. Rajendra Prasad Government Medical College (Dr. RPGMC) Tanda, India.

Patients and Methods: We analysed records of all children with KD below 15 years of age enrolled in Pediatric Rheumatology Clinic (PRC) at Dr. RPGMC, Tanda from 2018 to 2022. Diagnosis and treatment were based on American Heart Association criteria.

Results: 24 children were diagnosed with KD during this period. Of these, 16 (66.7%) were boys. Majority of cases 16 (66.7%) occurred in children between 1-5 years. Amongst these, 2/3 rd were diagnosed with incomplete KD. Coronary artery abnormalities (CAA) on echocardiography were noted in 8 (33.3%) children. Two children were given therapy in addition to IVIg in form of infliximab. Both of these patients were infants and had CAA at the time of presentation.

Discussion: Cell of Pediatric Clinical Immunology and Rheumatology was established in the department of Pediatrics at Dr. RPGMC, Tanda in 2020. Recognition of rheumatological conditions, including KD, has increased after this.

Conclusion: There is increase in number of cases of KD being recognized, possibly due to increased awareness about KD, improved diagnostic facilities and better imaging modalities. Presence of Pediatric Rheumatologist is an important factor in increased awareness and increased diagnosis of KD. Incomplete KD is more common than complete KD. Greater male preponderance correlates with other Indian studies.
Figure 1: Frequency of presenting manifestations of rheumatic diseases

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Table 1: Initial clinical features of children with mixed connective tissue disease fulfilling different criteria

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Table 2: Initial clinical features of adult versus pediatric onset mixed connective tissue disease

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Clinical spectrum of anti-MDA5 antibody associated juvenile dermatomyositis from a tertiary care centre in South-India

Murugan Sudhakar, K Anu Punnen, Dharshini Sathishkumar1, T Sathish Kumar; Departments of Pediatrics and 1Dermatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Anti–MDA5 autoantibody associated juvenile dermatomyositis (JDM) is characterized by clinically amyopathic form and rapidly progressive interstitial lung disease (RP-ILD). However, ethnic variations in disease manifestations have been noted.

Objectives: To describe the clinical spectrum of anti-MDA5 associated JDM from a tertiary-care hospital in South-India.

Methods: A single-centre retrospective analysis of anti-MDA5 associated JDM over a period of 5 years.

Results: Eight children had positive anti-MDA5 autoantibody out of 31 children with JDM (25.8 %) included in the study period. Mean age at onset of clinical manifestations and age at diagnosis are 7.1 years and 7.7 years respectively. Mean delay in diagnosis was 6.8 months. F:M ratio was observed to be 1:1.6. Mean follow-up period was 9 months (range 2-17 months). Classical cutaneous manifestations like heliotrope rash (n=3), and gottron papules (n=7), inverse gottron (n=2), and other rash (n=8), were identified in majority of them. All of them had muscle disease with severe truncal weakness (100%); pharyngeal and neck muscle involvement in 2 patients each. Arthritis was present in 50% of patients. Out of 8 patients, 6 patients showed radiological evidence of ILD (75%). Six of them had no significant respiratory symptoms and only 2 had symptomatic ILD in follow up, of which 1 succumbed due to RP-ILD. Most remained in remission with corticosteroids and Mycophenolate mofetil (n=5); corticosteroids and Methotrexate (n=3) with 2 required Rituximab for ILD. Remission was seen in 5 out of 8 patients within mean duration of 6 months.
Figure 2: Distribution of final diagnoses

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Conclusion: RP-ILD, commonly noted with anti-MDA5 associated dermatomyositis in South-East Asian population, is seen only in 25% our pediatric cohort of anti-MDA5, however, the frequency of ILD was more (75%) as like South-East Asian population. We also noted that significant proportion of children with anti-MDA5 JDM in our cohort had severe muscle disease and arthritis.


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Clinical profile of children with enthesitis related arthritis from a tertiary care centre in South India

Sowdagar Shama, Murugan Sudhakar1, T Sathish Kumar1, John Mathew, Ashish J Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, 1Department of Pediatrics, Division of Pediatric Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Enthesitis related arthritis (ERA), characterised by male predominance, later age of onset, enthesitis, axial disease and peripheral arthritis, is the most common category of juvenile idiopathic arthritis (JIA) reported from India.

Objectives: We aim to describe the demographic, clinical, laboratory and treatment profile of patients with ERA at their first visit.

Methods: Demographic, clinical, laboratory, imaging and treatment details of included patients, at their first visit to the paediatric rheumatology clinic at a tertiary care hospital, between July 2016 and June 2021 were retrieved from electronic medical records. All patients fulfilled the ILAR criteria.

Results: Of the 221 patients included, 199 (90%) were males. Demographics, clinical features and treatment details are highlighted in [Table 1]. Peripheral arthritis and axial arthritis were present in 160 (72.4%) and 86 (38.9%) patients, respectively. The most commonly involved peripheral joints were knees (60.2%) and ankles (39.8%). Enthesitis was observed in 68 (30.8%) patients, with achilles tendons being the most affected entheses (55, 80.5%) [Figure 1]. Uveitis (current/past) was documented in 13 (5.9%) patients. Median (IQR) CRP and ESR were 16.8 (5.15-44.4) mg/L and 40 (24-55) mm, respectively. Data on imaging for sacroiliitis (magnetic resonance imaging) and enthesitis (ultrasound) were available in 25% of patients. Sulphasalazine was the most commonly used conventional disease modifying anti-rheumatic drug (cDMARDs) (66.5%). Concomitant use of non-steroidal anti-inflammatory drugs was observed in 45.7% of patients. Inadequate response to cDMARDs led to treatment with biologic(b) DMARDs in 8 (3.6%) patients. Median time to initiate bDMARDs was 4 years [Figure 1].
Figure 1: Pattern and frequency of enthesitis in patients with ERA

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Conclusion: A high prevalence of HLA-B*27 in patients with ERA was noted. The lag period between onset of symptoms and diagnosis was less in this cohort. cDMARDs were the most commonly used drugs and use of bDMARDs was sparse.


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Study of macrophage activation syndrome in children with sepsis: A cross sectional study

Ashok Kannepalli, Anu Maheswari, Sunita Sharma, Deonath Mahto; Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India

Background: There is hidden underlying immune mediated mechanism in uncontrolled sepsis in terms of immune paralysis or hyperimmune response which is reflected as Macrophage activation syndrome.

Objective: To Study the prevalence of Macrophage activation syndrome in sepsis and comparison of IL6, Calprotectin, and various laboratory parameters in MAS and Non-MAS group.

Methods: A Cross sectional observational study was done by enrolling a total number of 105 cases grouped into sepsis, severe sepsis and septic shock( qualifying criteria SIRS +ORGAN DYSFUNCTION) and evaluated for qualifying criteria of MAS, IL-6 and calprotectin levels are assessed in cases with MAS and without MAS.

Results: Prevalence of MAS is 18% (19 cases) in the study group which accounted for 42.1% in severe sepsis and 57.9% in septic shock. The mean levels of IL-6 in MAS group was 96.97 and Non-MAS group is 88.29 which was not statically significant (P value- 0.074) and Calprotectin MAS group is 18889.16 (6995.62) and Non MAS group is 18045.08 (7455.78). which was not statiscally significant (p value-0.721). The Mean (SD) of Ferritin (mg/L) is 207.97, 548.25, and 1108.06 in Sepsis, Severe Sepsis and Septic Shock respectively which showed Significant Value when compared to the groups in study. There was a Moderate positive correlation between Ferritin (mg/L) and CRP (mg/L) which was statiscally significant which showed For every 1 unit increase in Ferritin (mg/L), the CRP (mg/L) increases by 0.02 units and Conversely, for every 1 unit increase in CRP (mg/L), the Ferritin (mg/L) increases by 2.24 units [Table 1].

Conclusion: MAS should be suspected in any case of uncontrolled sepsis which is indirect expression of MODS in sepsis. The need to develop risk stratification model for assessment of MAS and timely intervention of IVIG and Steroids for better outcome.


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Juvenile systemic lupus erythematosus related pancreatitis: A clinical and management conundrum

Sanjib Mondal, Anjani Gummadi, Deepti Suri, Anmol Bhatia1, Rakesh Kumar Pilania, Ankur Kumar Jindal, Pandiarajan Vignesh, Amit Rawat, Anju Gupta, Surjit Singh; Department of Paediatrics, Advanced Paediatrics Center, PGIMER, 1Department of Radiology, PGIMER, Chandigarh, India

Background: Pancreatitis is a rare but potentially life-threatening complication of juvenile systemic lupus erythematosus (jSLE). There are a few reports in the literature about this association, but the pathogenesis and treatment are still controversial. We report 6 children who developed acute pancreatitis in systemic lupus erythematosus.
Table 1: Details of demographics, clinical features and treatment in patients with enthesitis related arthritis (n=221)

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Materials and Methods: We retrospectively studied 295 children with jSLE who were registered in the Pediatric Rheumatology Clinic, Advanced Paediatrics Centre of the Post Graduate Institute of Medical Education and Research, Chandigarh from 1993 to 2021. We collected clinical, laboratory data, follow up and outcome of patients with jSLE who had pancreatitis.

Results: We diagnosed 295 children with SLE during the period 1993–2021. We have recorded 6 (2%) [3 boys] patients with pancreatitis during the study duration. Median age of onset of disease was 10 years (range 6-12 years). Of these, 5 had pancreatitis as initial manifestation of lupus and pancreatitis (acute abdomen) was the reason for medical attention in 4 children. Renal system was involved in all 6 (100%) patients. Three patients had mucocutaneous manifestations and one had neurological involvement. Anti-phospholipid antibody positivity was noted in 2 patients (33.3%), while one (16.6%) had macrophage activation syndrome. Children were successfully treated with pulse glucocorticoids (n=5), cyclophosphamide (n=2) and mycophenolate mofetil (n=2). Four children (66.6%) developed pseudo pancreatic cyst due to complication of acute pancreatitis. One child with positive APLA, neurological and renal involvement died after recurrent episodes of pancreatitis. Two patients (33.3%) developed recurrent episodes of pancreatitis during follow up [median 10.5 year (range 1.5-15 years)]. We noted mortality in 16.6% of the patients (n=1) in our present cohort.

Conclusion: SLE related pancreatitis should be considered when patients with lupus develop abdominal pain and vomiting. Earlier recognition and diagnosis can reduce overall morbidity and mortality.


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Chronic non-bacterial osteomyelitis: A study from a single centre in India

Anju Abraham1, Debasis Patro1, Jyothi Raghuram2,3, Anand Prahalad Rao1,3; 1Pediatric Rheumatology Clinic, Manipal Hospitals, 2Aster Whitefield Hospital, 3Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India.

Background: First described in 1972 by Giedion et al, Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disorder that presents as recurrent bone pain in one or multiple sites.
Figure 1: Correlation be1tween Ferritin (mg/L) and CRP (mg/L) (n = 105)

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Table 1: Association between macrophage activation syndrome and sepsis severity (n=105)

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Objectives: 1. To understand the clinical profile of CNO patients from the Indian subcontinent. 2. To elucidate the outcomes of the children affected with CNO.

Methods: We retrospective reviewed 38 cases diagnosed as CNO as per Bristol Diagnostic criteria in the Pediatric rheumatology clinic in Manipal hospital, Bengaluru, India after obtaining ethical approval.

Results: The findings are summarized in the [Table 1]. Of the 164 multifocal lesions detected by WB- MRI in 33 cases, tibial lesions (26%) were the commonest. LPIN2 deficiency was also reported in two cases. All cases were initiated with naproxen. 27 patients were started on methotrexate of which 2 cases each responded to Bisphosphonates and TNF inhibitor respectively. 4 out of 25 cases required additional TNF inhibitor after pamidronate and methotrexate therapy. 2 cases responded well to pamidronate infusion alone. 8 children had frequent flares. 14 children are in remission on drugs and 12 children are in remission off drugs. 2 cases are lost to follow up.

Conclusion: With increasing, knowledge and timely referral to a rheumatology center may bridge the gap to delay diagnosis. WB- MRI can detect bone lesions and prevents unnecessary invasive procedures. In a resource-limited setting, methotrexate and pamidronate have helped in achieving remission. TNF inhibitors are helpful in resistant cases with good response.

Keywords: Auto inflammatory bone disease, chronic non-bacterial osteomyelitis (CNO), chronic recurrent multifocal osteomyelitis (CRMO), LPIN, whole body magnetic resonance imaging (WB-MRI)

Table of Comparison:


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Effect of withholding methotrexate after vaccination with ChAdOx1 nCov19 in patients with inflammatory arthritis: Two parallel randomized controlled trials (MIVAC I and II)

U Rashwith1, Padmanabha Shenoy1,2, Teny Grace Skaria2, S Anu1, Sneha Joseph2, Sakir Ahmed3, Pankti Mehta4, Aby Paul2, Seena Elsa Oommen2, Justin George2, Manju Mohanan2; 1Sree Sudheendra Medical Mission, Kochi, 2Centre for Arthritis and Rheumatism Excellence, Nettore, Kerala, 3Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, 4King George Medical University, Lucknow, Uttar Pradesh, India

Background: optimal strategy balancing improved immunogenicity and risk of flare after holding methotrexate following COVID vaccination is still evolving.

Objectives: To compare the efficacy and safety of holding MTX after each (MIVAC 1) and only after the second dose (MIVAC II) of ChAdOx1 vaccine versus continuation of MTX in two RCTs.

Methods: Two parallel RCTs were conducted in patients with RA or PsA on stable doses of MTX. (CTRI reg. no. MIVAC I: CTRI/2021/07/03463 & MIVAC II: CTRI/2021/07/035307). In MIVAC I, patients were randomised to hold or continue MTX for two weeks after each dose of vaccine. MIVAC II included patients who had continued MTX during the 1st and were randomised to hold or continue MTX for 2 weeks after 2nd [Table 1]. Primary outcome of the trials was the anti-RBD antibody titres measured 4 weeks after the second dose. Secondary outcome was the flare rate, defined as increase in disease activity or physician intent to hike DMARDs.

Results: In MIVAC I, 250 participants were randomized and 158 completed the study. In MIVAC II, 178 participants were randomized and 157 completed the study. After analysis, even though median antibody titres were found significantly higher in MTX hold group compared to the control group in both trials, there was no difference in antibody titres between two MTX hold arms across two trials [Figure 1]. Flare rates were higher in hold group in MIVAC I, whereas there was no difference in the flare rates between the two groups in MIVAC II.

Conclusion: Holding MTX after both the doses or only after the second dose of ChAdOx1 yields higher anti-RBD antibody titres as compared to continuing MTX. Comparing across the trials, holding MTX only after the second dose appears to be non-inferior to holding MTX after both doses of the vaccine with a lesser risk of flare.


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PIMS-TS also known as MIS-C is a relatively new entity causing multisystem involvement in children and adolescents following SARS-CoV 2 infection

Koushik Sur, Debabrata Man, Suparna Guha; Vivekananda Institute of Medical Sciences, Kolkata, West Bengal, India

Background: Since the initial case series reports from UK various there have been reports pouring in from all across the globe. These studies are mainly based on the acute phase of the disease, but there is a paucity of data regarding the outcome of the disease.

Objectives: To study the clinical phenotype of PIMS -TS patients in the acute phase and analyse their short-term outcome at 6 months.

Methods: This study was conducted at the Department of Pediatric Medicine in VIMS. All patients less than 12 years of age admitted from March 2021 to September 2021 with PIMS-TS (fulfilling the WHO criteria) were included in the study. The data regarding the acute phase of the disease was obtained from the in-patient records. Follow-up was done by a multidisciplinary team for 6 months and the data was obtained from the hospital out-patient department.

Results: 25 patients (14 male, 11 female) were included in this study. Median age of presentation was 3 years. All patients had elevated markers of systemic inflammation at baseline. None of the patients died. By 6 months, systemic inflammation was resolved in all. The commonest presentation was fever (100%), rash (70%) followed by neurological (66%) and gastrointestinal (50%) manifestations. 17 patients had significant findings in Echocardiography. Coronary arterial dilatation seen in 10 patients & mild to moderate pericardial effusion in 10 patients. Complete heart block in ECG seen in 1 patient who needed permanent pacing. One patient had acute pancreatitis. Contrast enhanced CT scan of abdomen suggestive of necrotic pancreatitis with chronicity of 6/10. One patient was diagnosed with autoimmune haemolytic anemia. Most of them needed immunomodulation in the form of steroids, IVIG, and biologics. At 6 months follow up, majority had completely recovered. Significant dilatation of coronary artery persisted in only 2 patients. At 6 months the patient with complete heart block was doing well with the permanent pacemaker. The patient with acute pancreatitis & autoimmune haemolytic anemia was doing very well at 6months. 3 patients were lost to follow up. PedsQL responses at 6 months follow up revealed mild impairment by parental report most in emotional and psychosocial scale (5 out of 13 that means 38% in both). No patient was in severe impairment group.



Conclusion: Biochemical markers or inflammation resolved in almost all patients by 6 months. But functional impairment in form of psychological, scholastic and emotional impairment persist for a long time.


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Effects of the second dose of COVID-19 vaccines in patients with autoimmune rheumatic diseases with hybrid immunity – A prospective cohort study

Anu Sreekanth1, Sakir Ahmed2, Pankthi Mehta3, Padmanabha Shenoy1,4, Aby Paul4, Aparna4, Somy Cherian4, Kripesh Kumar4; 1Sree Sudheendra Medical Mission, Kochi, 2Centre for Arthritis and Rheumatism Excellence, Nettore, Kerala, 3Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, 4King George Medical University, Lucknow, Uttar Pradesh, India

Background: Hybrid immunity is a robust antibody response post vaccination in individuals with prior history of infection than in uninfected fully vaccinated subjects, being described in COVID 19. The effect of second vaccine dose on subjects with hybrid immunity is less studied.

Objective: determine the effect of second dose of COVID-19 vaccine between single versus double dose vaccinated individuals with AIRDs who were previously infected with COVID-19 (hybrid immunity).

Methods: Based on the number of vaccine doses (V) and order of occurrence of infection (I), AIRD patients previously infected with COVID-19, who had received at least one dose of AZD1222 (N=200) were stratified into four groups: I+V, I+V+V, V+I, V+V+I (50 each), Anti-RBD (Receptor Binding Domain) antibodies were compared between them. In 49 patients of the I+V+V group, who had received AZD1222, paired sera were compared for antibody levels and neutralization post-first & second dose. Controls were AIRD patients who had hybrid immunity after BVV152 (30)/ taken 2 doses of COVID-19 vaccines (14 AZD1222, 11 BBV152) but with no previous infection.

Results: The highest levels of anti-RBD antibodies were observed in the V+V+I group (18219±7702 IU/ml) with similar titres in the I+V+V (10392±8514 IU/ml) and the I+V (8801±8122 IU/ml). Thus, the second dose did not appear to boost antibody levels in those with hybrid immunity. This was confirmed in the 49 paired samples that paradoxically showed a lowering of antibody titres after the second dose [9626 (IQR: 4575-18785) to 5781 (2484-11906); p<0.001]. Delta neutralization was unaffected by the second dose however, that of Omicron was significantly reduced [45.7 (5.3 - 86.53) % to 35 (7.3-70.9) %; p=0.028].

Conclusion: The second dose of AZD1222 did not boost antibody titres in patients with AIRD who had previous COVID-19, instead led to lower antibody titres and neutralization of the Omicron variant.


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COVID-19 severity and vaccine breakthrough infections in idiopathic inflammatory myopathies, other systemic autoimmune and inflammatory diseases, and healthy Individuals: Results from the COVID-19 vaccination in autoimmune diseases study

Mrudula Joshi, Leonardo Santos Hoff1, R Naveen2, Samuel Katsuyuki Shinjo3, Jessica Day4, 5, 6, Parikshit Sen7, Jucier Gonçalves Junior8, James B. Lilleker9,10, Vishwesh Agarwal11, Sinan Kardes12, Minchul Kim13, Marcin Milchert14, Ashima Makol15, Tamer Gheita16, Babur Salim17, Tsvetelina Velikova18, Abraham Edgar Gracia-Ramos19, Ioannis Parodis20,21, Albert Selva O'Callaghan22, Elena Nikiphorou23,24, Ai Lyn Tan25,26, Tulika Chatterjee27, Lorenzo Cavagna28,29, Miguel A Saavedra30, Nelly Ziade31,32, Johannes Knitza33, Masataka Kuwana34, Arvind Nune35, Oliver Distler36, Döndü Üsküdar Cansu37, Lisa Traboco38, Suryo Angorro Kusumo Wibowo39, Erick Adrian Zamora Tehozol40, Jorge Rojas Serrano41, Ignacio García-De La Torre42, Chris Wincup43,44, John D Pauling45,46, Hector Chinoy47,48,49, Vikas Agarwal50, Rohit Aggarwal51, Latika Gupta52,53,54,55 and COVAD Study Group; Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, 11Mahatma Gandhi Mission Medical College, Navi Mumbai, Maharashtra, 2,50,52Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 7Maulana Azad Medical College, New Delhi, India, 1, 3, 8Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil, 4Department of Rheumatology, Royal Melbourne Hospital, 5Walter and Eliza Hall Institute of Medical Research, Parkville, 6Department of Medical Biology, University of Melbourne, Melbourne, Australia, 9,47,53Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, The University of Manchester, 48National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, The University of Manchester, Manchester, 10Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, 49Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, 23Centre for Rheumatic Diseases, King's College London, 24Department of Rheumatology, King's College Hospital, 43Department of Rheumatology, Division of Medicine, Rayne Institute, University College London, 44Centre for Adolescent Rheumatology Versus Arthritis at UCL, UCLH, GOSH, London, 25NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, 26Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, 35Southport and Ormskirk Hospitals NHS Trust, Southport, 45Royal National Hospital for Rheumatic Diseases, Royal United Hospitals, 46Department of Pharmacy and Pharmacology, University of Bath, Bath, 54Department of Rheumatology, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, 55City Hospital, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, United Kingdom, 12Department of Medical Ecology and Hydroclimatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, 37Department of Internal Medicine, Division of Rheumatology, Eskişehir Osmangazi University, Eskişehir, Turkey, 13,27Department of Internal Medicine, Center for Outcomes Research, University of Illinois College of Medicine Peoria, Illinois, 14Department of Internal Medicine, Rheumatology, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Szczecin, Poland, 15Division of Rheumatology, Mayo Clinic, Rochester, Minnesota, 51Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA, 16Department of Rheumatology, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt, 17Department of Rheumatology, Fauji Foundation Hospital, Rawalpindi, Pakistan, 18Department of Clinical Immunology, Medical Faculty, University Hospital “Lozenetz”, Sofia University St. Kliment Ohridski, Sofia, Bulgaria, 19Department of Internal Medicine, General Hospital, National Medical Center “La Raza”, Instituto Mexicano del Seguro Social, 30Departamento de Reumatología, Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, IMSS, 41Rheumatologist and Clinical Investigator, Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Mexico City, 42Departamento de Inmunología y Reumatología, Hospital General de Occidente and University of Guadalajara, Guadalajara, Jalisco, Mexico, 20Department of Medicine Solna, Division of Rheumatology, Karolinska Institutet and Karolinska University Hospital, Stockholm, 21Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden, 22Department of Internal Medicine, Vall D'hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, 40Rheumatology, Medical Care and Research, Centro Medico Pensiones Hospital and Instituto Mexicano del Seguro Social Delegación Yucatán, Yucatán, Spain, 28Department of Rheumatology, Fondazione I.R.C.C.S. Policlinico San Matteo, 29Dipartimento di Medicine Interna e Terapia Medica, Rheumatology Unit, Università Degli Studi Di Pavia, Pavia, Italy, 31Department of Rheumatology, Saint-Joseph University, 32Department of Rheumatology, Hotel-Dieu de France Hospital, Beirut, Lebanon, 33Medizinische Klinik - Rheumatologie Und Immunologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Deutschland, 34Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 36Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 38Philippine Rheumatology Association, St Luke's Medical Center, Bonifacio Global City, Philippines, 39Department of Internal Medicine, Rheumatology Division, Fakultas Kedokteran, Universitas Indonesia, Jakarta, Indonesia

Background: Significant gaps are present in the evidence of the spectrum and severity of COVID-19 infection in idiopathic inflammatory myopathies (IIM). The possibility of attenuated immunogenicity and reduced efficacy of COVID-19 vaccines due to concomitant immunosuppressive medication is a major concern in these patients, and there is little data available on COVID-19 vaccine breakthrough infections (BI) in IIM.
Figure 1

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Figure 2: Effect of second does in those previous COVID-19 infection

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Objectives: We aimed to compare the spectrum and severity of COVID-19 and vaccine breakthrough infections (BIs) among patients with IIMs, other systemic autoimmune and inflammatory diseases (SAIDs), and healthy controls (HCs).

Methods: This is a cross-sectional, self-reported online global survey (CoVAD survey) that collected demographics, COVID-19 infection, and vaccination details from April-September 2021. Adult patients with at least one COVID-19 vaccine dose were included. BIs were defined as infections occurring >two weeks after any dose of vaccine. Characteristics associated with BI were analysed with a multivariate regression analysis.

Results: Among 10,900 respondents [42 (30-55) years, 74%-females, 45%-Caucasians] HCs were (47%), SAIDs (42%) and IIMs (11%). Patients with IIMs reported fewer COVID-19 cases before vaccination (6.2%-IIM vs 10.5%-SAIDs vs 14.6%-HC; OR=0.6, 95%CI 0.4-0.8, and OR=0.3, 95%CI 0.2-0.5, respectively). BIs were uncommon (1.4%-IIM; 1.9%-SAIDs; 3.2%-HC), and occurred in 17 IIM patients, 13 of whom were on immunosuppressants, and 3 (18%) required hospitalization. All-cause hospitalization was higher in patients with IIM compared to HCs [23 (30%) vs 59 (8%), OR=2.5, 95%CI 1.2-5.1 before vaccination, and 3 (18%) vs 9 (5%), OR=2.6, 95%CI 1.3-5.3 in BI]. In a multivariate regression analysis, age 30-60 years was associated with a lower odds of BI (OR=0.7, 95%CI 0.5-1.0), while the use of immunosuppressants had a higher odds of BI (OR=1.6, 95%CI 1.1-2.7) [Table 1].

Conclusions: Patients with IIMs reported fewer COVID-19 cases than HCs and other SAIDs, but had higher odds of all-cause hospitalization from COVID-19 than HCs. BI were associated with the use of immunosuppressants and were uncommon in IIMs.


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Optimizing timing for the third dose of the AZD1222 vaccine in patients with autoimmune rheumatic diseases

Anu Sreekanth1, Sanjana Joseph2, Sakir Ahmed3, B Gayathri2, S Anandu2, Aby Paul2, Padmanabha Shenoy1,2; 1Sree Sudheendra Medical Mission, Kochi, 2Centre for Arthritis and Rheumatism Excellence, Nettore, Kerala, 3Department of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: Autoimmune rheumatic disease (AIRD) patients are at higher risk for COVID19, hence they are recommended to take a third dose of COVID vaccination. AZD1222 (“Covishield”) is the mostly widely used vaccine in India.

Objectives: To estimate the optimal time for the third dose of AZD1222.

Methods: Patients with AIRD who had received 2 doses of ADZ1222 previously, and also ADZ1222 as the third dose were included. Anti-RBD antibody levels were estimated 4 weeks after the second and third doses each. A successful 'boost' was defined as at least 50% rise of antibody titres with the absolute titre >212 IU/mL. Generalized linear modelling (GLM) was used to estimate optimal strategies for the third dose.

Results: 302 patients with AIRD [Age: 53 years (IQR: 45-60); 238 (78.8%) females] had post-second dose titres of 3746.0 IU/mL (1371.5-7243.5) that increased to 6103.0 IU/mL (3209.0- 10503.0) post-third dose [p<0.001]. A GLM model showed no association between boosting effect and time interval between 2nd and 3rd dose (after adjustment for pre-booster antibody titres). However, another GLM showed that the interval between 3rd dose and the last event (vaccine dose/infection) was significantly associated with the boosting effect along with age, sex, antibody titres after 2nd dose, and methotrexate and tofacitinib doses. The boosting effect was negligible in the 3-6 months period after the last event, with no statistically significant difference between 6-9 months and more than 9 months periods [Table 1].

Conclusions: Maximum boosting effects were seen in patients who had received the third dose at least 6 months after the last event. Other factors affecting the boost were age, sex, use of methotrexate and tofacitinib; but not presence of hybrid immunity, underlying AIRD, use of low dose steroids, hydroxychloroquine or sulfasalazine.


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Clinical presentations, severity, and outcomes in inflammatory rheumatic diseases patients receiving conventional and/or biologic DMARD's during first wave of COVID-19 pandemic: A single centre study in United Kingdom

Amol Sagdeo, Ayman Askari, Robert Jones, Raghvendra Prasad Patil, Lisa Burgess, Yan Herman Kuiper; Robert Jones Agnes Hunt Orthopaedic Hospitals, NHS Foundation Trust, Oswestry, United Kingdom

Background: In the first wave of Covid-19 pandemic, it was very difficult to know how many patients with IRD's were affected due to Covid-19, how severe were their clinical manifestations and what were their outcome due to loss of clinic contact due to remote working and lockdowns particularly in United Kingdom.
Figure 1: (a) Flow diagram of study participants (b) Symptomatology of COVID-19 infection in IIM prior to vaccination compared to BI (c) Symptomatology of COVID-19 infection prior to vaccination in IIMs compared to other SAIDs (d) Symptomatology prior to vaccination in IIMs compared to HCs. IIMs: Idiopathic Inflammatory Myopathies, SAIDs: Systemic Autoimmune Inflammatory Diseases, HCs: Healthy Controls, BI: Vaccine Breakthrough COVID-19 Infection

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Table 1: Univariate and multivariable regression analysis of characteristics associated with COVID -19 breakthrough infection

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Table 2

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Objectives: The aim of this study was to understand various clinical manifestations of Covid-19 in IRD patients and to know their clinical outcomes in patients registered in our rheumatology outpatients' clinics receiving disease modifying agents.

Methods: All patients registered with our rheumatology clinic on conventional and biologic DMARD's were contacted by a postal survey regarding their covid symptoms and outcomes on 20th October 2020 and data was then collected over next 3 months. The data collected included patients' demographics, diagnosis, medication, comorbidities, their covid test results and if they needed hospital admission. Out of 1311 surveys sent out 610 were returned and were analysed.

Results: A total of 610 patients' data was analysed. The mean age of participants in this study was 63.86 years (SD 13.30). The rheumatic diagnosis of the study population (N=610) was RA 350 (57.4%), PsA76 (12.5%), AS73 (12.0%), OP40 (6.6%), RA+OA38 (6.2%), OA13 (2.1%), Other 7 (1.1%), JIA6 (1.0%), RA+OP6 (1.0%). Patients who were shielding were 361 (61.9%) out of 610. Covid symptoms observed in this study Sore throat in 109 (18.4%), New continuous cough in 43 (7.3%), High temp in 41 (7.0%), Loss/change taste in 41 (6.9%), Loss/change smell in 32 (5.4%), New muscle/joint ache in 106 (18.0), New breathlessness 55 (9.4). Out of 610 patients in the study 58 (9.6%) patients needed hospital admission 58 (9.6%) and 14 (2.3%) patient did not fill the box for admission. 12 (2%) patients replied yes to the question,' have you had a diagnosis of Covid-19, of which only 2 had a positive Covid test, 5 patients tested negative, and 5 patients did not reply. Out of 12 patients who were diagnosed as having covid-19, seven had rheumatoid arthritis (2 of these were tested positive for PCR) and 5 had psoriatic arthritis. None of the patients in this study died or needed invasive ventilation.
Figure 2

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Conclusions: Inflammatory rheumatic disease patients receiving disease modifying agents did not demonstrate excess risk of mortality or have risks of serious covid-19 disease in our study.


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Efficacy of an additional dose of vector vaccine (AZD1222) versus inactivated (BBV152) vaccine in patients with autoimmune rheumatic diseases previously having inadequate seroconversion: Randomized controlled trial

Anuroopa Vijayan, Aswathy Sukumaran, Sara Jones1, Sakir Ahmed2, Pankti Mehta3, Aby Paul, Manju Mohanan, Sageer, Padmanabha Shenoy; Dr Shenoy's Care Clinic, Kochi, 1Rajiv Gandhi Centre for Biotechnology, Thiruvananthapuram, Kerala, 2Kalinga Institute of Medical Sciences, Bubaneshwar, Odisha, 3King George's Medical University, Lucknow, Uttar Pradesh, India

Background: An additional dose of COVID-19 vaccine is being offered to vaccinated people, especially to those who are immunocompromised. In India, the most widely available vaccines are the adenoviral vector-borne AZD1222 (ChAdOx1 nCoV-19) and the heat-inactivated (BBV152). This study compared the efficacy between these two vaccines in patients with autoimmune rheumatic diseases (AIRD).
Table 1: Generalized linear modelling for successful boost of antibody titres after the 3rd dose of the vaccine

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Objectives: To compare final anti-SARS-CoV-2 antibody titres, neutralization of pseudovirions by these antibodies and T cell responses between patients of AIRD who had received the third dose of AZD1222 and BBV152 vaccines.

Methods: Patients with stable AIRD who had completed two doses of COVID-19 vaccination but had suboptimal response (anti-RBD antibody <212) were randomized (1:1) to receive either AZD1222 or BBV152 as a booster dose. Patients with previous hybrid immunity or those who developed COVID-19 during the trial were excluded. Antibody titres, neutralization of Wuhan and Omicron pseudovirions and interferon release by T cells (ELISPOT) in response to the Spike antigen were measured four weeks after this booster dose. [Trial registration: CTRI/2021/12/038928].

Results: 146 were screened, 91 randomized and 67 analysed per protocol. The third dose [Figure 1] improved antibody titres [p<0.001], neutralization of Wuhan strain [p<0.001], and T cell interferon release [p<0.001] but not neutralization of the Omicron strain [p=0.24]. Antibody titres were higher (p<0.005) after ADZ1222 boost [2414IU (IQR: 330-10315)] than BBV1222 [347.7IU (0.4-973)]. Neutralization of the Wuhan strain was better [AZD1222: 76.6% (23.0-95.45) versus BBV152:32.7% (0-78.9), p=0.03 by ANCOVA]. Neutralization of Omicron [0 (0-28.4) vs 0 (0-4.8)] and T cell interferon release [57.0 IU (23.5-95) vs 50.5 (13.2-139)] were similar. The effects of the booster stratified by the primary vaccine used are presented in [Figure 2].
Figure 1: (a-c) Antibody titres after 2nd and 3rd doses of ADZ1222 when the third dose was given between 12-24 weeks (a), 24-36 weeks (b) or after 36 weeks (c). D: Comparison of the boost of antibody titres (pre and post 3rd dose) for these three time frames

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Conclusion: The third dose improved all parameters of immunogenicity in AIRD patients with previous inadequate responses except Omicron neutralization. The vector born vaccine appears to be superior at least in terms of antibody titres and Wuhan strain neutralization.


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Vaccine hesitancy among patients with idiopathic inflammatory myopathies and rheumatic diseases in 2021-2022: A comparative analysis of COVID-19 vaccination in autoimmune diseases surveys

R Naveen, Mrudula Joshi1, Parikshit Sen2, Vishwesh Agarwal3, Samuel Shinjo4, Sinan Kardes5, James B Lilleker6,7, Hector Chinoy6, 8, 9, Ashima Makol10, Oliver Distler11, Minchul Kim12, Elena Nikiphorou13,14, Ai Lyn Tan15,16, Babur Salim17, Tamer A Gheita18, Nelly Ziade19,20, Tsvetelina Velikova21, Tulika Chatterjee22, Arvind Nune23, Marcin Milchert24, Ioannis Parodis25,26, Abraham Edgar Gracia-Ramos27, Albert Selva O'Callaghan28, Miguel Saavedra29, Lorenzo Cavagna30,31, Masataka Kuwana32, Johannes Knitza33, Jessica Day34,35,36, Carlos Enrique Toro Gutiérrez37, Carlo Caballero38, Dzifa Dey39, Erick Adrian Zamora-Tehozol40, Jorge Rojas Serrano41, Ignacio Garcia-De La Torre42, Iris Colunga43, Javier Merayo-Calico44, John Pauling45,46, Chris Wincup47,Armen Yuri Gasparayan48, Vikas Agarwal,Rohit Aggarwal49,Latika Gupta1, 7, 50,51; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 1Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, 3Mahatma Gandhi Mission Medical College, Navi Mumbai, Maharashtra, 2Maulana Azad Medical College, New Delhi, India, 4Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil, 5Department of Medical Ecology and Hydroclimatology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey, 6Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, The University of Manchester, 8National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, The University of Manchester, Manchester, 7Neurology, Manchester Centre for Clinical Neurosciences, Northern Care Alliance NHS Foundation Trust, 9Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, 13Centre for Rheumatic Diseases, King's College, 14Department of Rheumatology, King's College Hospital, 46Department of Rheumatology, University College, London, 15NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, 16Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, 23Southport and Ormskirk Hospital NHS Trust, Southport, 44Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), 45Department of Pharmacy and Pharmacology, University of Bath, Bath, 48Departments of Rheumatology and Research and Development, Dudley Group NHS Foundation Trust, Russells Hall Hospital, Clinical Research Unit, Dudley, 50Department of Rheumatology, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton, 51City Hospital, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, United Kingdom, 10Division of Rheumatology, Mayo Clinic, Rochester, Minnesota, 12Department of Internal Medicine, Center for Outcomes Research, University of Illinois College of Medicine Peoria, 22Department of Internal Medicine, Center for Outcomes Research, University of Illinois College of Medicine Peoria, Illinois, 49Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA, 11Department of Rheumatology, University Hospital Zürich, University of Zürich, Zürich, Switzerland, 17Department of Rheumatology, Fauji Foundation Hospital, Rawalpindi, Pakistan, 18Department of Rheumatology, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt, 19Department of Rheumatology, Saint-Joseph University, 20Department of Rheumatology, Hotel-Dieu de France Hospital, Beirut, Lebanon, 21Department of Clinical Immunology, Medical Faculty, University Hospital “Lozenetz”, Sofia University St. Kliment Ohridski, Sofia, Bulgaria, 24Department of Internal Medicine, Rheumatology, Diabetology, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Szczecin, Poland, 25Department of Medicine Solna, Division of Rheumatology, Karolinska Institutet and Karolinska University Hospital, Stockholm, 26Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden, 27Department of Internal Medicine, General Hospital, National Medical Center “La Raza”, Instituto Mexicano del Seguro Social, 29Departamento de Reumatología Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, IMSS, 41Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, 43Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición “Salvador Zubirán”, Mexico City, 40Centro Médico Pensiones, Autoimmunity Division, Mérida, Yucatán, 42Rheumatology Service, Facultad de Medicina Y Hospital Universitario “Dr. JoseE. Gonzalez”, Universidad Autonoma de Nuevo Leon, Monterrey, Mexico, 28Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Vall D'hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, Spain, 30Department of Rheumatology, Fondazione I.R.C.C.S. Policlinico San Matteo, 31Dipartimento di Medicine Interna e Terapia Medica, Rheumatology Unit, Università Degli Studi Di Pavia, Pavia, Italy, 32Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 33Medizinische Klinik 3 - Rheumatologie und Immunologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Deutschland, 34Department of Rheumatology, Royal Melbourne Hospital, 35Walter and Eliza Hall Institute of Medical Research, 36Department of Medical Biology, University of Melbourne, Parkville, Australia, 37Centro de Referencia en Osteoporosis, Reumatología and Dermatología, Cali, 38Department of Medicine, Universidad del Norte, Barranquilla, Colombia, 39Department of Medicine and Therapeutics, Rheumatology Unit, University of Ghana Medical School, College of Health Sciences, Korlebu Teaching Hospital, Accra, Ghana

Background: COVID-19 vaccine hesitancy has been influenced by many unknown factors despite its efficacy in improving morbidity and hospitalization following SARS-CoV-2 infection.

Objective: We aimed to analyze the factors influencing vaccine hesitancy in 2022 and compare them with those in 2021 through multicentre international e-surveys (The COVID-19 Vaccination in Autoimmune Diseases Studies-COVAD study).

Methods: COVAD-19 vaccine hesitancy was studied is a global patient reported COVAD e-survey [Figure 1]a.
Figure 1: COVID diagnosed participants

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Figure 1: Effect of third dose vaccine

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Results: Among 18882 (2021) and 7666 responses (2022), and 16.5% and 5.1% did not receive COVID-19 vaccine respectively. The prevalence of vaccine hesitancy has decreased [OR 0.26]. In 2022, among vaccine non-recipients IIMs 69 (17.8%), AIRDs 179 (46.3%), other AIDs 80 (20.6%), and HC 59 (15.2%). The reasons were long-term safety concerns 152 (39.2%), awaiting more safety data 105 (27.1%), not believing in the science behind vaccine 79 (20.4%), doctor advising against it 47 (12.1%), and not recommended due to recent infection 30 (7.8%). IIM patients comprised more disbelievers of the vaccine than AIRDs and HCs patients [OR 1.8, p=0.023 AIRDs, OR 4, p<0.001 HC], had more long-term safety concerns [OR 1.9, p=0.001 AIRDs, OR 5.4, p<0.001 HC] and had more doctors recommending against the vaccine [OR 12.9, p<0.001 HC] [Figure 1]b. Vaccine non-recipients had higher pain visual analog score (VAS), lower fatigue VAS, lower PROMIS10a physical health and lower mental health scores [Table 1]. Factors predicting vaccine hesitancy were lower PROMIS10a global physical health score [OR=0.9] and Caucasian ethnicity [OR=4.2]. Compared to 2021, doctor's advising against vaccination [OR=2.5] and long-term safety concerns [OR=3.6] were more frequent causes, whereas vaccine non-availability [OR=0.05] and have scheduled the vaccination but not received [OR=0.1] were less frequent causes in 2022 [Figure 1]c.
Figure 2: The effects of the booster stratified by the primary vaccine

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Table 1: Disease modifying anti-rheumatic drugs therapy received by the participating subjects (by brands)

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Conclusions: Even though the prevalence of vaccine hesitancy has decreased, long-term safety concerns is a major reason for vaccine hesitancy.


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Evaluation of immune response following SARS–CoV-2 vaccination using COVAXIN in systemic lupus erythematosus patients on immunosuppressive therapy

Angan Karmakar, Dipendranath Ghosh, Parasar Ghosh; Department of Clinical Immunology and Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Objective: To evaluate seroreactivity after COVID-19 vaccination using COVAXIN in patients with systemic lupus erythematosus (SLE) who are on immunosuppression.

Methods: Thirty SLE patients and twenty healthy controls receiving a complete COVID-19 vaccine regimen were using COVAXIN were included. SLE patients with history of major organ involvement were included and they were on immunosuppressive medications along with oral corticosteroid and hydroxychloroquine. IgG seroreactivity to the SARS–CoV-2 spike protein were measured at baseline, after two weeks and after four months of completion of two doses of vaccination.

Results: Fully vaccinated SLE patients produced significantly lower IgG antibodies against SARS–CoV-2 spike protein compared to fully vaccinated controls. Among SLE patients, ten patients (33%) did not produce significant IgG antibody level two weeks after the complete vaccination and among healthy controls, that was in only one subject (5%). After four months of the second dose of vaccine significant anti SARS-CoV2 IgG antibody produced in six (20%) SLE patients and one (5%) healthy subject. Though SARS-CoV2 IgG level was more in healthy controls after 2weeks and 4 months of complete vaccination (semiquantitatively measured optical density ratio 4.14±2.11 and 6.99±3.14 respectively) than SLE patients on immunosuppression (3.03±2.82 and 5.29±3.74), the results were not statistically significant.

Conclusion: SLE patients, on immunosuppression, has lower IgG response to spike protein of SARS-CoV2 after complete vaccination with COVAXIN, than healthy subjects. Majority of SLE patients achieved significant anti SARS-CoV2 IgG antibody level after four months of complete vaccination. In spite of immunosuppressive medication, two doses COVAXIN has the ability to produce significant immune response in SLE patients.


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Flares following COVID-19 Vaccination in patients with idiopathic inflammatory myopathies: Combined analysis from the COVAD studies

R Naveen, Zolten Gringer, Vishwesh Agarwal, Mrudula Joshi, Parikshit Sen, Samuel Katsuyuki Shinjo, Sinan Kardeş, James B Lilleker, Ashima Makol, Oliver Distler, Elena Nikiphorou, Ai Lyn Tan; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 2Mahatma Gandhi Mission Medical College, Navi Mumbai, 3Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, Maharashtra, 4Maulana Azad Medical College, New Delhi, India, 1Department of Medicine, Division of Clinical Immunology, University of Debrecen, Debrecen, Hungary

  1. Sinan Kardeş- Department of Medical Ecology and Hydroclimatology, Istanbul Faculty of Medicine, Istanbul University, Capa-Fatih, 34093, Istanbul, Turkey. Orcid ID: 0000-0002-6311-8634. [email protected]
  2. James B Lilleker- Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK; Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, Salford, UK. Orcid ID: 0000-0002-9230-4137. [email protected]
  3. Ashima Makol- Division of Rheumatology, Mayo Clinic, Rochester, MN, USA. Orcid ID: 0000-0002-8748-898X. [email protected]
  4. Oliver Distler- Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland. Orcid ID: 0000-0002-0546-8310. [email protected]
  5. Elena Nikiphorou- 1. Centre for Rheumatic Diseases, King's College London, London, UK. 2. Rheumatology Department, King's College Hospital, London, UK. Orcid ID: 0000-0001-6847-3726. [email protected]
  6. 6. Ai Lyn Tan- 1. NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK. 2. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. Orcid ID: 0000-0002-9158-7243. [email protected]
  7. Babur Salim- Rheumatology Department, Fauji Foundation Hospital, Rawalpindi, Pakistan. Orcid ID: 0000-0001-8430-9299. [email protected]
  8. Tamer A Gheita- Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt. Orcid ID: 0000-0002-1155-9729. [email protected]
  9. Nelly Ziade- 1. Rheumatology Department, Saint-Joseph University, Beirut, Lebanon. 2. Rheumatology Department, Hotel-Dieu de France Hospital, Beirut, Lebanon. Orcid ID: 0000-0002-4479-7678. [email protected]
  10. Tsvetelina Velikova- Department of Clinical Immunology, Medical Faculty, University Hospital “Lozenetz”, Sofia University St. Kliment Ohridski, 1 Kozyak Str., 1407, Sofia, Bulgaria. Orcid ID: 0000-0002-0593-1272. [email protected]
  11. Tulika Chatterjee, Department of Internal Medicine, University of Illinois College of Medicine at Peoria, Illinois. Orcid ID: 0000-0001-8844-851X. [email protected]
  12. Carlos Enrique Toro Gutiérrez- Centro de Referencia en Osteoporosis, Reumatología & Dermatología, Cali, Colombia
  13. Carlo Caballero-Department of Medicine, Universidad del Norte, Barranquilla, Colombia
  14. Dzifa Dey- Rheumatology Unit, Department of Medicine and Therapeutics, Korlebu Teaching Hospital, University of Ghana Medical School, College of Health Sciences, Korle-Bu, Accra, Ghana
  15. Erick Adrian Zamora-Tehozol- Centro Médico Pensiones, Autoimmunity Division, Mérida, Yucatán, Mexico
  16. Jorge Rojas Serrano- Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico
  17. Ignacio Garcia-De La Torre- Departamento de Inmunología y Reumatología, Hospital General de Occidente, Guadalajara, Jalisco, Mexico
  18. John Pauling- Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), Upper Borough Walls, Bath, BA1 1RL, UK; Department of Pharmacy and Pharmacology, University of Bath, Bath, UK
  19. Chris Wincup- Department of Rheumatology, University College London, London, UK
  20. Dr Arvind Nune- Southport and Ormskirk Hospital NHS Trust, Southport, PR8 6PN, UK. Orcid ID: 0000-0002-3849-614X. [email protected]
  21. Marcin Milchert- Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, ul Unii Lubelskiej 1, 71-252, Szczecin, Poland. Orcid ID: 0000-0002-0943-8768. [email protected]
  22. Ioannis Parodis- 1. Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden. 2. Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. Orcid ID: 0000-0002-4875-5395. [email protected]
  23. Abraham Edgar Gracia-Ramos- Department of Internal Medicine, General Hospital, National Medical Center “La Raza”, Instituto Mexicano del Seguro Social, Av. Jacaranda S/N, Col. La Raza, Del. Azcapotzalco, C.P. 02990 Mexico City, Mexico. Orcid ID: 0000-0003-1842-2554. [email protected]
  24. Albert Selva-O'Callaghan- Systemic Autoimmune Diseases Unit, Internal Medicine Department, Vall D'hebron General Hospital, Universitat Autonoma de Barcelona, 08035 Barcelona, Spain. Orcid ID: 0000-0003-2823-9761. [email protected]
  25. Miguel A Saavedra- Departamento de Reumatología Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, IMSS, MexicoCity, Mexico. Orcid ID: 0000-0003-0687-9944. [email protected]
  26. Lorenzo Cavagna- 1. Department of Rheumatology, Fondazione I.R.C.C.S. Policlinico San Matteo, Pavia, Italy. 2. Rheumatology Unit, Dipartimento di Medicine Interna e Terapia Medica, Università degli studi di Pavia, Pavia, Lombardy, Italy. Orcid ID: 0000-0003-3292-1528. [email protected]
  27. Masataka Kuwana- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8602, Japan. Orcid ID: 0000-0001-8352-6136. [email protected]
  28. Johannes Knitza- Medizinische Klinik3 - Rheumatologie und Immunologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Ulmenweg18, 91054, Erlangen, Deutschland. Orcid ID: 0000-0001-9695-0657. [email protected]
  29. Jessica Dey- 1. Department of Rheumatology, Royal Melbourne Hospital, Parkville, VIC 3050, Australia. 2. Walter and Eliza Hall Institute of Medical Research, Parkville, VIC 3052 Australia. 3. Department of Medical Biology, University of Melbourne, Parkville, VIC 3052 Australia. Orcid ID: 0000-0001-8528-4361. [email protected]
  30. Hector Chinoy- 1. Division of Musculoskeletal and Dermatological Sciences, Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, Manchester, UK. 2. National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, The University of Manchester, Manchester, UK. 3. Department of Rheumatology, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK. Orcid ID: 0000-0001-6492-1288. [email protected]
  31. Vikas Agarwal- Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. Orcid ID: 0000-0002-2089-027X. [email protected]
  32. Rohit Aggarwal- Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA. Orcid ID: 0000-0001-7531-8038. [email protected]
  33. Latika Gupta- 1. Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. 2. Dept of Rheumatology, New Cross Hospital, Royal Wolverhampton Trust, Wolverhampton, WV10 0QP, United Kingdom. Orcid ID: 0000-0003-2753-2990. [email protected]


Background: Flares following COVID-19 vaccination are an emerging concern among patients with rare rheumatic disease like idiopathic inflammatory myositis (IIMs), whereas data and understanding of this is rather limited.

Aim: We aimed to study the prevalence, characteristics and determinants of flare of IIM following COVID-19 vaccination.

Methods: CoVAD (COVID-19 Vaccination In Autoimmune Diseases) surveys are global patient self-reported e-survey from 109 countries conducted in 2021 and 2022. Flares of IIM were defined by 4 definitions; a. patient self-reported, b. physician and immunosuppression (IS) denoted, c. sign directed (new erythematous rash, or worsening myositis or arthritis), d. MCID worsening of PROMISPF10a score between the patients who had taken both the surveys. Descriptive stats and multivariate regression were used to describe the predictors of flare. Cox-regression analysis was used to differentiate flares by IIM subtypes.

Results: Among the 1278 IIM patients, aged 63 (50-71) years, 276 (21.5%) were dermatomyositis, 237 (18.5%) IBM, 899 (70.3%) were females and most were Caucasians (80.8%). Flares of IIM were seen in 123/1278 (9.6%), 163/1278 (12.7%), 112/1278 (8.7%), and 16/96 (19.6%) by definitions a-d respectively with median time to flare being 71.5 (10.7-235) days. Muscle weakness (69.1%), and fatigue (56.9%) were the most common symptoms of flare [Table 1]. The predictors of self-reported flare were: active/worsening disease prior to first dose of vaccine (OR=10.1, 95%CI=4.3-23.6), and anxiety disorder (OR=2.2, 95%CI=1.1-4.7). Rituximab use (OR=0.3, 95%CI=0.1-0.7) and IBM (OR=0.3, 95%CI=0.1-0.7) were protective. Physician defined flares were seen more often in females, mixed ethnicity, and those with asthma, ILD, and anxiety disorder (OR ranging 1.6-7.0, all p<0.05). Notably, overlap myositis (OM) had higher HR for flare compared to polymyositis (HR=2.3, 95%CI=1.2-4.4, p=0.010) [Figure 1].
Figure 1: (a) Methods (b) Various causes of vaccine hesitancy (*p<0.05, **P<0.005, ***p <0.001), (c) Comparison of causes of vaccine hesitancy among COVAD 1 and 2 surveys (*p<0.05, **P<0.005, ***p <0.001)

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Table 1: Comparison of baseline characteristics of vaccine recipients and non -recipients (The COVID-19 vaccination in autoimmune diseases 2 survey)

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Conclusion: Nearly one in ten individuals with IIM develop flares after vaccination, more so among women, those with overlap myositis, and active disease prior to vaccination.


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Impact of routine follow up care of rheumatology patients due to COVID 19 pandemic, a cross sectional single centre study from South India

Ashvin Rajeev Pillai, Kishan Venugopal, Arun Tiwari, Ishit Agarwal, Harsha Jacob, Vishal Marwaha; Amrita School of Medicine, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: This study was aimed to assess the patient's perspective of the impact of the COVID-19 pandemic on routine follow-up care of rheumatic diseases as there is a paucity of literature in this topic.
Table 1: Characteristics of idiopathic inflammatory myositis patients with flare (defined by 4 definitions)

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Objectives: To assess the impact of Covid 19 on follow up and management of Rheumatology patients. To study the efficacy and awareness of newer modalities of consultation among Rheumatology patients in Covid 19 pandemic.

Methods: This was a cross-sectional study conducted at a single tertiary care center in South India. Patients with rheumatic diseases on long-term follow-up were enrolled and a predesigned questionnaire was administered to assess the impact of the COVID-19 pandemic on their follow up care.

Results: Totally 277 were enrolled, 127 (45.8%) continued office visits for follow-up and 103 (37%) used telemedicine and 27 (9.7%) lost to follow-up completely due to pandemic. Telemedicine was not known to 187 (67.5%) patients and 65 (20.1%) were satisfied with telemedicine. Partial loss of follow-up was reported by 146 (52.7%) and 72 (25.9%) patients developed complications due to loss of a regular follow-up. Unavailability of medicines in time was reported by 49 (17.6%) cases. COVID-19 pandemic had caused anxiety in 83 (29.9%) cases.

Conclusion: According to our study, the COVID-19 pandemic-imposed challenges in terms of loss of follow-ups, unavailability of medicines in time, increased anxiety and novel changes like telemedicine were accepted by the majority of the patients.


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Predictors of COVID – 19 severity and outcomes in Indian patients with rheumatic diseases: A prospective cohort study

Background: It is generally believed that patients with rheumatic and musculoskeletal disorders (RMDs) have greater chances of being infected with COVID-19 and resulting adverse outcomes and mortality. There is dearth of data regarding COVID-19 outcomes amongst RMD patients from South-East Asia.
Figure 1: Cox-regression analysis of time to flare among IIM subtypes. OM had higher HR for flare than PM [HR 2.3 (1.2-4.4), p 0.010]

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Objectives: This study was conducted to assess the demographic and clinical characteristics of COVID-19 infection along with predictors associated with adverse outcomes- hospitalisation, advanced respiratory support and mortality amongst a cohort of RMD patients under follow up in a tertiary rheumatology clinic in northern India.

Methods: This prospective cohort study included RMD patients infected with RT-PCR-confirmed COVID-19 between April 2020-October 2021. Demographic and clinico-laboratory details of both COVID-19 and underlying RMD were described. Predictors of mortality, hospitalization, and severe COVID-19 were identified using stepwise multivariable logistic regression analyses.

Results: A total of 64 COVID-19 infected RMD patients [median age 41.5 (19-85 years); 46 (72%) females] were included. 18 (28%) patients had severe COVID-19. 23 (36%) required respiratory support [11 (17%) of these required mechanical ventilation]. 36 (56%) patients required hospitalization [median duration of stay 10 (1-42) days]; 17 (27%) required ICU admission. Presence of comorbidities [OR=4.5 (1.4-14.7)] was an independent predictor of COVID-19 severity. Comorbidities [OR=10.7 (2.5-45.4)] and SLE [OR=7.0 (1.2-40.8)] were independently associated with hospitalization. Ongoing rheumatic disease activity [OR=6.8 (1.3-35.4)] and underlying diagnosis of SLE [OR=7.1 (1.2-42.4)] and SSc [OR=9.5 (1.5-61.8)] were found to be strong independent predictors of mortality in RMD patients infected with COVID-19. Age, gender, underlying CTD-ILD and choice of immunomodulatory/ immunosuppressive therapy were not associated with COVID-19 outcomes in Indian RMD patients.

Conclusion: Presence of comorbidities was independently associated with COVID-19 severity and hospitalization. Ongoing rheumatic disease activity and presence of SLE or SSc independently predicted mortality. Age, gender, type of immunomodulatory/ immunosuppressive therapy and presence of CTD-ILD did not affect COVID-19 outcomes in Indian RMD patients.


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COVID vaccination and flare of autoimmune rheumatic disorders

S Bhavana, Vijaya Prasanna Parimi, R N Tejaswini, G Narsimulu; Department of Rheumatology, ESIC Medical College, Hyderabad, Telangana, India

Background: COVID-19 disease caused by the SARS-CoV-2 virus is an ongoing pandemic. Vaccination is proven to be an effective measure for protection from severe disease and mortality in COVID 19. The dilemma around safety of vaccination in patients with autoimmune/ autoinflammatory rheumatic diseases is the most common cause of vaccine hesitancy in patients.

Objectives: We aimed to study use of vaccines in patients with AIRDs, occurrence of disease flare and interactions with immunosuppressive drugs.

Methods: Consecutive patients over a period of 4 months, with Autoimmune disease who visited Rheumatology department OPD and had disease flare or new onset disease after COVID vaccination were studied.

Results: A total of 40 patients had disease flare after receiving COVID vaccine. The most common underlying AIRD was Rheumatoid arthritis in 60% patients followed by Psoriatic arthritis (20%), and lupus (7%). Most of the patients had mild disease (50%) or remission (42.5%) prior to vaccine intake. Mild flare was observed in 12.5% post vaccination, Moderate flare in 72.5% and Severe flare in 15% with a mean duration of 11.6 days after vaccine. It took an additional 15.5 days on average, to control the flare of disease by either increasing dose of steroids or DMARD therapy.

Conclusions: Individuals who were in remission prior to vaccine intake, had a lower chance of flare after vaccination. Those having mild or moderate disease activity had severe flares after vaccination. Parameters like age, gender, type of vaccine did not have a significant effect.


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Analysis of SARS-COV-2 antibodies in patients with autoimmune rheumatic diseases with no prior history of COVID-19 infection

Debanjali Sinha, Sulagna Chatterjee1, Sumantro Mondal2, Sanchaita Mishra3, Shyamashis Das4, Alakendu Ghosh3; Institute of Neurosciences, Departments of 1Clinical Immunology and Rheumatology and 3Rheumatology, Institute of Post-Graduate Medical Education and Research, Kolkata, West Bengal, India

Introduction: Little data is known about the seroprevalence of antibodies to SARS-CoV-2 in patients with rheumatic diseases who are on Disease modifying anti-rheumatic drugs and/or immunosuppressives.

Objectives: To evaluate the titer of SARS-CoV-2 antibodies in patients with autoimmune rheumatic diseases with no prior history of COVID infection and compare it with healthy controls.

Methods: We collected sera from patients suffering autoimmune diseases, specifically Rheumatoid arthritis (RA) and Systemic Lupus Erythematosus (SLE), who have no prior history of COVID infection. Sera were also collected from healthy controls, who were family members of the respective patients, without any past history of COVID infection. We tested the titre of COVID antibodies in both patients and healthy controls. Two types of antibodies were tested – antibodies to the nucleocapsid protein (NP) and antibodies to the receptor binding domain (RBD) of the spike (S) protein of SARS-CoV-2. Data collection was done between the first and second waves of SARS-CoV2 in India, before vaccination.

Results: A total of 62 patients were included in the study, of which 35 patients were SLE and 27 RA. There was no statistically significant difference in the NP and RBD antibody levels between patients with autoimmune diseases and healthy controls (Mean RBD 49.09 vs 62.04 in HC, p 0.0575 and mean NP 0.95 vs 1.68 in HC, p 0.0876). Antibodies to the RBD of the spike protein of SARS-CoV-2 were significantly low in patients with SLE as compared to healthy subjects (Mean 19.25 vs 56.23 in HC, p 0.0180) whereas no such difference was seen in RA patients (p 0.4813). No significant difference was found in antibody levels between patients on steroids versus those not on steroids.

Conclusion: Patients of SLE were found to have significantly low spike protein antibodies as compared to normal population. This may predispose them to COVID infection.


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Post COVID-19 auto-immune rheumatic diseases and outcome: A tertiary care centre experience

Danveer Bhadu, Shah Alam1, Rudra P Goswami, Uma Kumar; Departments of Rheumatology and 1Orthopaedics, All India Institute of Medical Sciences, New Delhi, India

Introduction: Several studies have reported the presence of autoantibodies in patients with coronavirus disease 2019 (COVID-19) in different frequencies: antinuclear antibodies (ANA) in 35.6%, anti-Ro/SSA in 25%, rheumatoid factor in 19%, lupus anticoagulant in 11% and antibodies against interferon (IFN)-I in 10%. The clinical spectrum of autoimmune-related manifestations in COVID-19 patients ranges from organ-specific (cutaneous vasculitis) to systemic autoimmune and inflammatory conditions (e.g., systemic vasculitis, myositis, SLE etc).

Objective: The aim of this study was to know association of various types of AIRDs after COVID-19 infection and their prognosis.

Methodology: Consecutive, previously healthy, post-COVID-19 patients with history of musculoskeletal problems were subjected for autoimmune screening tests (RF, Anti-CCP, ANA By IIF, ANCA by IIF, ENA by LIA and HLA-B27). Those patients who fulfilled the inclusion and exclusion criteria were enrolled in this study.
Figure 1

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Inclusion Criteria: History of symptomatic COVID-19 infection confirmed by RTPCR test. Auto-Immune Rheumatic Diseases (AIRDs) symptoms within 2 months of symptomatic COVID-19 infection. Patient fulfilling criteria of AIRDs as per existing classification or diagnosed by expert rheumatologist.

Exclusion Criteria: Any symptoms suggestive of AIRD prior to COVID-19 infection.

Results: Total 15 patients who had evidence of AIRDs were enrolled in this study. Male to female ratio was 8:7. Five patients fulfilled the criterion for rheumatoid arthritis (RA), 2 patients for granulomatosis polyangiitis (GPA), 2 patients for axial spondyloarthritis SpA), one each for dermatomyositis, possible Behchet's disease, urticarial vasculitis, seronegative arthritis, nonspecific tenosynovitis and enthesitis. All patients responded well with conventional treatment regime except one GPA patient who initially responded to induction therapy but had relapse after 6 months and succumbed to his illness [Table 1].
Table 1: Baseline demographic and clinical data of patients with rheumatic and musculoskeletal diseases included in the study

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Table 2: Stepwise univariable and multivariable logistic regression analysis for predictors of mortality in patients with rheumatic diseases infected with COVID -19

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Conclusion: Almost every type of AIRDs can be triggered by COVID-19 infection and most of them have good response with conventional treatment regime.


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Characteristics of post-COVID-19 in autoimmune rheumatic diseases: Data from COVID-19 vaccination in autoimmune diseases study

R Naveen1, S Shaharir2, Parikshit Sen3, Mrudula Joshi4, Vishwesh Agarwal5, Samuel Katsuyuki Shinjo6, Sinan Kardeş7, James B Lilleker8,9, Ashima Makol10, Oliver Distler11, Ai Lyn Tan12,13, Elena Nikiphorou14,15, Babur Salim16, Tamer A Gheita17, Nelly Ziade18,19, Tsvetelina Velikova20, Tulika Chatterjee21, Carlos Enrique Toro Gutiérrez22, Carlo Caballero23, Dzifa Dey24, Erick Adrian Zamora-Tehozol25, Jorge Rojas Serrano26, Ignacio Garcia De La Torre 27, John Pauling28,29, Chris Wincup30, Arvind Nune31, Marcin Milchert32, Ioannis Parodis33, Abraham Edgar Gracia-Ramos34, Albert Selva-O'Callaghan35, Miguel A Saavedra36, Lorenzo Cavagna37,38, Masataka Kuwana39, Johannes Knitza40, Jessica Dey41,42, Hector Chinoy43,44,45, Vikas Agarwal1, Rohit Aggarwal46, Latika Gupta1,47; 1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 4Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, 5Mahatma Gandhi Mission Medical College, Navi Mumbai, Maharashtra, 3Maulana Azad Medical College, New Delhi, India, 2Department of Internal Medicine, Universiti Kebangsaan Malaysia Medical Centre, Cheras Kuala Lumpur, Malaysia, 6Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, Brazil, 7Department of Medical Ecology and Hydroclimatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, 8Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, The University of Manchester, 45Division of Musculoskeletal and Dermatological Sciences, Centre for Musculoskeletal Research, School of Biological Sciences, The University of Manchester, 46National Institute for Health Research Manchester Biomedical Research Centre, Manchester University NHS Foundation Trust, The University of Manchester, Manchester, 9Manchester Centre for Clinical Neurosciences, Salford Royal NHS Foundation Trust, 47Department of Rheumatology, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, 30Southport and Ormskirk Hospital NHS Trust, Southport, 12Centre for Rheumatic Diseases, King's College London, 29Department of Rheumatology, University College London, 13Department of Rheumatology, King's College Hospital, London, 13NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, 14Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, 49Department of Rheumatology, New Cross Hospital, Royal Wolverhampton Trust, Wolverhampton, United Kingdom, 10Division of Rheumatology, Mayo Clinic, Rochester, 48Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA, 11Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 15Department of Rheumatology, Fauji Foundation Hospital, Rawalpindi, Pakistan, 16Department of Rheumatology, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt, 17Department of Rheumatology, Saint-Joseph University, 18Department of Rheumatology, Hotel-Dieu de France Hospital, Beirut, Lebanon, 19Department of Clinical Immunology, Medical Faculty, University Hospital “Lozenetz”, Sofia University St. Kliment Ohridski, Sofia, Bulgaria, 20Department of Internal Medicine, University of Illinois College of Medicine at Peoria, Illinois, 21Centro de Referencia en Osteoporosis, Reumatología and Dermatología, Cali, 22Department of Medicine, Universidad del Norte, Barranquilla, Colombia, 23Department of Medicine and Therapeutics, Rheumatology Unit, Korlebu Teaching Hospital, University of Ghana Medical School, College of Health Sciences, Korle-Bu, Accra, Ghana, 24Centro Médico Pensiones, Autoimmunity Division, Mérida, Yucatán, 25Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias, 34Department of Internal Medicine, General Hospital, National Medical Center “La Raza”, Instituto Mexicano del Seguro Social, 36Departamento de Reumatología Hospital de Especialidades Dr. Antonio Fraga Mouret, Centro Médico Nacional La Raza, IMSS, Mexico City, 26Departamento de Inmunología y Reumatología, Hospital General de Occidente, Guadalajara, Jalisco, Mexico, 27Royal National Hospital for Rheumatic Diseases (at Royal United Hospitals), 28Department of Pharmacy and Pharmacology, University of Bath, Bath, 31Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Poland, 32Department of Medicine Solna, Division of Rheumatology, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, 33Department of Rheumatology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden, 35Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Vall D'hebron General Hospital, Universitat Autonoma de Barcelona, Barcelona, Spain, 38Department of Rheumatology, Fondazione I.R.C.C.S. Policlinico San Matteo, Pavia, 39Dipartimento di Medicine Interna e Terapia Medica, Rheumatology Unit, Università Degli Studi Di Pavia, Pavia, Lombardy, Italy, 40Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 41Medizinische Klinik 3 - Rheumatologie und Immunologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Deutschland, 42Department of Rheumatology, Royal Melbourne Hospital, 43Walter and Eliza Hall Institute of Medical Research, 44Department of Medical Biology, University of Melbourne, Parkville, Australia

Background: Post COVID-19 is the most persistent and challenging issue in the peri-pandemic period.
Figure 2

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Figure 3

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Figure 1: Scleritis in granulomaosis polyangiitis [Figure 1]a and Behcet's Disease patients respectively

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Aim: We aimed to assess the prevalence, characteristics of this syndrome in those with autoimmune rheumatic diseases (AIRDs) through a multicentre international e-surveys (The COVID-19 Vaccination in Autoimmune Diseases) CoVAD study.

Methods: The COVAD study, is a global patient reported e-survey with collaborators from 109 countries, conducted in Jan-July 2022. The survey captured COVID-19 infection, vaccination data AIRDs, other non-rheumatic autoimmune diseases (nrAIDs), and healthy controls (HCs). Post COVID-19 was defined (WHO definitions) as persistence of symptoms beyond 3 months of COVID-19 infection. Descriptive statistics and multivariable regression were employed.

Results: Among 1677 who had taken the survey >90 days of last COVID-19 infection 8.1% (n=136) had post COVID-19 [aged 46 (34-55) years, Male:Female=1:6.3]. The prevalence of post COVID-19 was 10.7% (n=81/755) in AIRDs, 8.1% (17/209) in other nrAIDs, and 5.3% (38/713) in HCs. The prevalence was higher in AIRDs compared to HC [OR-2.1 (1.4-3.1),p=0.002] [Table 1]. The predictors of post COVID-19 in the entire cohort were females, Caucasian ethnicity, presence of fatigue, muscle aches, dyspnoea, loss of taste, and insomnia (during last COVID-19 infection). The use of rituximab, iv immunoglobulins (IVIG), and mycophenolate mofetil use also predicted post COVID-19 [Table 2]. The predictors in AIRDs were presence of any comorbidities [OR-2.6 (1.3-5.1), p=0.006] and mental health disorders [OR-2.1 (1.03-4.4), p=0.040]. Those with post-COVID-19 had worser PROMIS10a global physical health score (p<0.001), global mental health score (p<0.001), fatigue VAS (p<0.001), and pain VAS (p<0.001) compared to those without post-COVID-19. They also reported higher flares of AIRDs following COVID-19 infection (OR-4.3).
Table 1: Summary of main reported cases of new onset auto-immune rheumatic diseases after COVID -19 infection

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Conclusions: The overall prevalence of post-COVID-19 was 8.1% with higher prevalence in AIRDs compared to HCs. The predictors included female gender, Caucasian ethnicity, presence of fatigue, myalgia, dyspnoea, loss of taste during last COVID-19 infection and use of rituximab, IVIG, and mycophenolate mofetil.


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Rheumatological manifestations of COVID19: An observational study in a tertiary care centre in Eastern India

Abhinaba Datta, Udas Chandra Ghosh; Department of General Medicine, Kolkata Medical College, Kolkata, India

Background: COVID-19 patients often experience fever, fatigue, muscle pain, or arthralgia. Viruses can be direct etiologic agents of acute and chronic arthritis and of different forms of vasculitis. The new virus outbreak represents a previously unseen differential diagnosis to be henceforth taken into consideration. These rheumatological manifestations can also prevail in patients post discharge at follow up. No data is available for such study is Eastern India.

Objectives: To diagnose different rheumatological manifestations of COVID19 and to calculate its prevelance separately. To asses disease flare in patients with pre-existing rheumatological disease infected with COVID19.

Methods: COVID RTPCR positive patients admitted in hospital and those attending post COVID follow up clinic were interviewed with a well-structured questionnaire and examined clinically. Patients with pre-existing rheumatological disease were examined for disease flare up. ESR, Serum CRP and NLR ratio were obtained. Additional investigations were done on case to case basis.

Results: Total 1000 patients with COVID RTPCR positivity were interviewed and examined 86 patients (8.6%) had rheumatological manifestations. Among 86 patients 14 (16%) patients had previous rheumatological disease. Most common symptom was myalgia in 52%. Out of 21 patients with joint pain predominantly involving large joints (70%), oligoarticular in 71%. In subgroup of patients with previous rheumatological disease 5 patients of Rheumatoid arthritis out of 8 i.e.62% had a disease flare 2 patients were newly diagnosed with rheumatoid arthritis. 1 patient was diagnosed with Multisystem Inflammatory Syndrome in Adults (MIS-A).
Table 1: Comparison of those with and without post-COVID-19 syndrome

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Table 2: Predictors of post-COVID-19 syndrome among the entire cohort (n=1677)

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Conclusions: To conclude, COVID19 can present with rheumatological manifestations during period of infection and also in the post infectious period and may warrant further investigations. Myalgia, fatigue and joint pain were predominant symptoms. COVID can cause disease flare in patients with RA. COVID can even accerelate any asymptomatic underlying autoimmune process.


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Comparison of temporal variation of prevalence of positive antinuclear antibody amongst organ donors during after the second COVID wave – Findings from a retrospective single centre study from a tertiary hospital in Kerala

Anand Sasidharan, Sandeep Surendran; Department of Clinical Immunology and Rheumatology, AIMS, Kochi, Kerala, India

Background: The autoimmune phenomenon has been described in many case reports associated with COVID-19 infections. However, it is unknown whether this infection can lead to serological autoimmunity. The presence of a positive antinuclear antibody could be an indicator for the development of future auto-immune diseases.

Objectives: To compare the seroprevalence of a positive antinuclear antibody in a hospital-based cohort of solid organ donors in two times periods-one during the second COVID-19 wave in 2021(January-July 2021) versus a similar study period in the following year after the post-COVID-19 wave in 2022(January-July 2022).

Methods: This was a retrospective cohort analysis of the incidence of ANA positivity by immune-fluorescence (IF) in healthy organ donors between the above-mentioned periods. All individuals involved in the study were adult population of aged more than 18 years. IF was performed on the serum of these patients at a dilution of 1:80 using as per manufacture directions (BIORAD) and was reported as a positive test for all intensities more than 1+ A total of 155 healthy donors were tested. Of these, 74 samples were tested in 2021 and 81 in 2022. Data were described using descriptive statistics and group comparison was made using the Chi-Square test.

Results: Of the 155 healthy donors, the mean age was 41.2 +/- 9.59 years. Out of these were 69 (44.5%) males and 86 (55.4%) females. Out of the 74 samples tested in 2021, 40 showed ANA positivity (54%) whereas out of the 81 samples tested in 2022, 29 showed ANA positivity (36%, p-value of 0.033).

Conclusions: There was a statistically significant difference in the prevalence of a positive ANA among the organ donor during the covid wave and after the covid wave. The clinical significance of this was not known and we need further follow-up studies to ascertain whether it is a temporary phenomenon or of clinical significance.


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Effect of additional dose of AZD1222 in autoimmune rheumatic disease patients with hybrid immunity

Anu Sreekanth1, Sanjana Joseph2, B Gayathri2, S Anandu2, Aby Paul2, Padmanabha Shenoy1,2; Sree Sudheendra Medical Mission, 2Centre for arthritis and Rheumatism Excellence, Kochi, Kerala, India

Background: Hybrid immunity is a robust antibody response post vaccination in individuals with prior history of Covid 19 infection. The effect of booster dose on subjects with hybrid immunity is less studied.

Objective: The objectives were to assess the effectiveness of booster dose in subjects with hybrid immunity and to determine the ideal time for boosting hybrid immunity.

Methodology: Based on the number of vaccine doses (V) and order of occurrence of infection (I), AIRD patients previously infected with COVID-19, who had received both dose of AZD1222 were stratified into four groups: I+V+V, V+I+V, V+V+I, V+V. Control arm included only patients who were SARS CoV 2 N protein seronegative at baseline whereas patients who showed seroconversion were considered as, V+V+Asymptomatic infection. All enrolled patients were vaccinated with booster dose of AZD1222. Base line Anti RBD (Receptor Binding domain) before booster dose of vaccination and Post Anti-RBD antibodies were estimated after, one month.

Results: 301 patients were recruited. Post booster dose of vaccination there were significant rise in Anti RBD antibodies in all these cohorts expect in V+V+Asymptomatic infection group (P =0.270) [Figure 1]. Patients who received their booster dose with 3-6 month of their last immune hit also didn't show significant boosting effect (P=120) [Figure 2]. Subgroup analysis showed that there was boosting effect in both hybrid and control arms who took vaccine at an interval of greater than 6 months. But there was no significant boosting effect in 3–6-month interval (P =0.157) in hybrid cohorts. Binary logistic regression model showed boosting between 9-6, 6-3 months from last immune hit, Methotrexate and steroid use were associated with poor boosting response.

Conclusion: Booster dose in patients especially with hybrid immunity has to be timed between a minimum interval of 6 months after last immune hit to acquire minimal boosting effect.


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Efficacy of latent tuberculosis infection screening and treatment in patients receiving biological disease-modifying antirheumatic drugs

Harsh Jain, S Kartik, Abhishek Kumar, Vaibhavi Velangi, Gargi Sasmal; Department of Rheumatology, Army Hospital Referral and Research, New Delhi, India

Background: Biological Disease-Modifying Anti-Rheumatic Drugs (bDMARDs) are being increasingly used in patients with various autoimmune rheumatic diseases (AIRD). There is an increased risk of developing tuberculosis (TB), mostly through reactivation of latent tuberculosis infection (LTBI), especially in patients who are on Tumour Necrosis Factor (TNF) inhibitors. Various guidelines recommend screening of LTBI for before anti-TNF treatment.
Table 1

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Objectives: To study the efficacy of LTBI screening in patients receiving bDMARDs.

Methods: The study included patients with any AIRD who were planned for treatment with bDMARDs. LTBI screening was done by Mantoux test using five tuberculin unit (TU) strength and/or QuantiFERON-TB Gold (QFTG) test. Those with a Mantoux test reading of ≥10 mm induration at 48–72 h and/or a positive QFTG test, were treated for LTBI with 3 month regimen of Rifampicin (R) and Isoniazid (H) and bDMARD was started 4 weeks after starting LTBI treatment. Patients were followed and monitored for features of TB and side effects of LTBI treatment.

Results: 341 patients were screened and treated with bDMARDs between July 2020 to July 2022 (Infliximab- 125, Rituximab- 87, Adalimumab- 37, Golimumab- 35, Etanercept- 23, Tocilizumab- 20, Secukinumab- 14). 51 patients (14.95%) were positive for LTBI. Incidence of TB in LTBI group was 1.96% (1 in 51) and 1.37% (4 in 290) in non-LTBI group and the difference was not statistically significant (p value: 0.557). Out of the 5 who developed tuberculosis, four patients were on Infliximab and one received adalimumab after failure of infliximab (All 5 patients had spondyloarthritis). Only three patients (5.8%) developed side effects on 3HR regimen of which one discontinued LTBI treatment.

Conclusion: Tuberculosis can develop even in LTBI negative patients who are on TNF inhibitors and hence require regular monitoring. 3HR regimen is well tolerated with few adverse events.


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Prevalence of HCQ associated cutaneous hyperpigmentation in rheumatic diseases and its risk factors

Prudhvi Krishna Manyam, C B Mithun, Diya Baju Parappat, Achanya Sankar, Anjaly S Nair1; Departments of Clinical Immunology and Rheumatology and 1Biostatistics, AIMS, Kochi, Kerala, India

Background: Hydroxychloroquine (HCQ) is an antimalarial agent widely used in rheumatology due to its immunosuppressive and anti-inflammatory properties. Although cutaneous hyperpigmentation is a well-known complication of HCQ, the data on the same in the Indian population is scarce.
Figure 1: Comparison of anti RBD antibodies in different hybrid cohorts and in control (infection naïve)

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Figure 2: Comparison of anti RBD antibodies based on time interval (from last immune hit to booster dose)

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Objectives: The objectives of the study are to estimate the prevalence of HCQ associated hyperpigmentation in rheumatic diseases and to find risk factors associated with it.

Methods: A cross-sectional study was conducted over a period of 6 months from February 2020 to September 2020 in the rheumatology department at AIMS, Kochi. 430 patients with rheumatic diseases who were currently on HCQ for at least 3 months were included in the study. A structured questionnaire was used to collect the demographic data. All patients were screened for cutaneous hyperpigmentation by an experienced and qualified rheumatologist. The Chi-square test was used as a test of significance for qualitative data. p value <0.005 was considered statistically significant. To estimate the risk factors of HCQ induced hyperpigmentation, multiple binary logistic regression was used.

Results: In this study, a total of 430 patients (378 female and 52 male) with rheumatic diseases were included. Amongst them 177 patients had HCQ induced hyperpigmentation. The mean age of the patients was 45.5 (±15.2) years. Hyperpigmentation was more commonly noted with a single dose regimen than divided dose regimen and with a cumulative dose of more than 200 grams. A significant association was also observed with the concomitant use of medications like Mycophenolate Mofetil and Azathioprine.

Conclusions: HCQ induced cutaneous hyperpigmentation is not a rare side effect, with a prevalence of 41.1% in the current study. Hence all patients requiring HCQ should be counselled for potential risk of cutaneous hyperpigmentation.

Univariate analysis -Comparison of patients with HCQ induced pigmentation and patients without HCQ- induced pigmentation.

Multivariate analysis -Comparison of patients with HCQ induced pigmentation and patients without HCQ- induced pigmentation.


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Cost reduction by lengthening inter dose duration verses efficacy; A prospective cohort study in Indore

Vivek Kattet, Yamuna Agrawal1, Neetu Gupta2, Sourabh Malviya3; Medanta Superspeciality Hospital, 3Department of Rheumatology and Clinical Immunology, Medanta Superspeciality Hospital, 2Department of Anaesthiology, MGM Medical College, Indore, Madhya Pradesh, India, 1Department of Pathology, BP Koirala Institute of Health Sciences, Dharan, Nepal

Background: TNF alpha blockers are the most effective drugs against Spondyloarthritis (SpA). Recommended dose schedule by ACR and EULAR is associated with better clinical outcome however with higher cost and theoretical risk of latent infection activation and increased likelihood of antibodies development against biological DMARDs (bDMARDs). The objectives of the study was to study the cost effectiveness of bDMARDs by increasing the gaps between two doses in Indian subcontinent.

Method: It was a prospective study carried among 50 SpA patients for 2 years. SpA was diagnosed according to Assessment in SpondyloArthritis international Society (ASAS) criteria. Initially ACR recommended dose of Adalimumab or Eternacept was given for 24 weeks. Among patients with clinical remission 25% dose of the bDMARDs was reduced after every 12 weeks by increasing gaps between two injections. The dose reduction by 25% was further continued every 12 weeks among patients who had clinical remission.

Result: The mean and median age was 28 years ± 6.2 years and 36 years respectively. Clinical presentation as low back pain, peripheral arthritis, enthesitis, dactylitis and uveitis was noted in 96%, 68%, 62%, 18% and 12% respectively. The baselines mean BASDAI, mean sobers and CRP were 7.2, 4.1 cm and 29 mg/L. By the end of 52 weeks all patients were on bDMARDS with mean BASDAI, mean sobers and CRP were 2.8, 6.7 cm and 3.9 mg/L. Post dose reduction the effectiveness of bDMARDs was maintained 96%, 90%, 84% and 66% at 36, 48, 60 and 72 weeks respectively. The maximum net cost reduction was observed up to third attempt (upto 60 weeks) with reduction of 37% and 42% in Adalimumab and Eternacept arm respectively. Further attempt of cost reduction by duration extension was associated with drop in cost benefit due to increase in relapses proportion. The dose reduction was stopped after 72 weeks due to increase in cost [Table 1].

Conclusion: Increasing gaps between two doses of bDMARDs only under clinical supervision among SpA can be a cost-effective option.




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Safety of DMARDs in patients of connective tissue disease and spondyloarthritis with hepatitis B surface antigen positivity

Shyam Shah, Ruchika Goel, Ashish J Mathew, Josna Joseph, John Mathew; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Patients who are HBsAg positive and with rheumatological diseases like Rheumatoid arthritis (RA), Spondyloarthritis (SpA), SLE and other connective tissue disease (CTD) are treated with DMARDs. There has been concern on drugs that will be safe in patient with HBsAg positivity. Limited data is available in this area.

Objectives: To find safety of DMARDs in Rheumatology patients who are HBsAg positive.

Methods: Retrospective data of patients who are treated with DMARDs for RA, SpA, SLE and other CTDs with HBsAg positivity was analysed. This was taken from the electronic medical records of 2007 to 2021. There were 147 patients. We looked at the safety of various DMARDs in HBsAg positivite patients in terms of effect on liver enzymes and viral DNA.

Results: Four out of 147 patients had flare of Hepatitis B after DMARDs administration,Flare of viral hepatitis had HBV DNA Viral load ≥ 10000 copies and transaminitis ≥ 4 times upper limit of normal. All 4 were on Methotrexate and Suplhasalazine. One on steroids and HCQ in addition.12 of 14 (85%) patients of RA on Methotrexate and 20 of 22 (90%) of SpA on Methotrexate didn't have transaminitis on follow up. Patients with SLE, UCTD and Sjogrens were few but didn't have transaminitis or viral load increase on follow up.

Conclusion: 2.72 % of patients had a flare of Hepatitis B after using DMARDs. 85% of patients with RA and 90 % of patients with SpA did not have flare of hepatitis on follow up in our study.


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A retrospective study on efficacy and safety of rituximab therapy in extrarenal SLE

John Kumar Das, John Mathew; Department of Clinical Immunology and Rheumatology, CMC Vellore, Tamil Nadu, India

Background: B cells have critical roles in the pathogenesis of SLE, providing a strong rationale for the study of targeted treatments that modify the effects of B cells on immunity. In our centre, we have 8-9 years' experience of using Rituximab therapy in lupus patients.

Objective: The current study was aimed at evaluating the efficacy and safety of Rituximab therapy in extrarenal lupus patients who were poorly responsive to conventional immunosuppression.

Methods: We retrospectively collected data for all patients treated with Rituximab for extrarenal indications from the electronic medical records at our center from January 2013 to December 2021. Indications of Rituximab therapy, background medications, disease activity measures and follow up data upto 1 year were scrutinized.

Results: 104 patients were admitted during the study period with a female predominance 96 (92.3%). Rituximab was administered for various indications [Table 1], hematological indication being the most common. Background steroid dose was reduced by 50% after administration of the 2nd dose of Rituximab. Follow up: 74 patients for 1 year, 13 for 6M & 4 for 3M & 13 patients were lost to follow up (LTFU). Repeat Rituximab infusions were required for 8 & 5 patients after 6 months & 1 year respectively. All patients were on HCQ, steroid and one second line- MMF (75), Azathioprine (8), Methotrexate (3), Tacrolimus (5) & Cyclosporine (3). Rituximab was given as only 2nd line therapy in 13 patients. 88 patients were vaccinated prior with pneumo/influvac. Non-serious infections occurred in 6 patients (Cellulitis, respiratory & urinary), one with herpes zoster & febrile neutropenia. Efficacy of Rituximab is shown in [Table 1] and [Table 2].
Table 1: Cost effectiveness verses efficacy of tumour necrosis factor blockers

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Table 2: Efficacy outcomes

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Conclusion: Rituximab can be a promising biologic agent which has shown efficacy as an effective treatment for patients with active SLE who are nonresponsive to standard immunosuppressive therapy and it is safe provided the patients are appropriately vaccinated.


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Assessment of methotrexate induced adverse effects in patients with rheumatoid arthritis

Ritika Tambe, Sania Gore, Asawari Raut, Pravin Patil; BVDU Pune, Pune Rheumatology, Centre, Pune, Maharashtra, India

Background: Methotrexate (MTX) is the most commonly used disease modifying drug patients with rheumatoid arthritis (RA).

Objectives: To study the pattern of MTX induced adverse effects (AEs) in patients with RA.

Materials and Methods: A prospective study was conducted over a span of 6 months at a single center in the outpatient rheumatology clinic. RA patients who experienced AEs with use of MTX were included in the study. All patients were above 18 years of age and have been taking MTX for more than 3 months. All relevant data was collected from patient's electronic medical records stored in Healthlink software.

Results: Out of 154 RA patients included in the study, majority patients were females (84%) with a median age of 48 yrs (IQR 35-65) and 16% were male with a median age of 55 yrs (IQR 45-75). The average gap between MTX intake and occurrence AEs was 3 months. The dose at which maximum AEs occurred was 15 mg. Majority patients (86%) were prescribed MTX orally and the rest 14% received MTX in the form of injections. There were no significant comorbidities associating with intolerance. The most common AE hair loss (55%) was followed by nausea (27%), mouth ulcers (13%) and transaminitis (5%). The issues related to MTX induced AEs resolved in 73% of the patients by reduction in dose and 27% patients required discontinuation of MTX.

Conclusion: The adverse effects with MTX use include nausea, vomiting, mouth ulcers, transaminitis with hair fall being the most prevalent AE. These are more commonly observed in females. AEs induced by MTX are dose dependent hence majority of AEs can be addressed by dose reduction. Nearly 30% patients required discontinuation of MTX due to AEs.
Figure 1: Disease and DMARDs use

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Figure 2: Disease wise distribution

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Clinical effects of LTBI screening and treatment in patients receiving biological disease-modifying antirheumatic drugs

Harsh Jain, S. Kartik, Abhishek Kumar, Vaibhavi Velangi, Gargi Sasmal; Department of Clinical Immunology and Rheumatology, Army Hospital Referral and Research, New Delhi, India

Background: Tumor necrosis factor (TNF) inhibitors play important role in achieving low disease activity in patients with rheumatic diseases. Despite the effectiveness, there is an increased risk (1.7 to 9 times) of developing tuberculosis (TB), mostly through reactivation of latent tuberculosis infection (LTBI) which is more common in endemic countries like India.

Objectives: To study the clinical effects of LTBI screening and treatment in patients receiving biological disease-modifying antirheumatic drugs (bDMARDs).

Methods: The study included patients who were planned and treated with bDMARDs after LTBI screening with Mantoux test using five tuberculin unit (TU) strength and QuantiFERON-TB Gold (QFTG) test. Those with a Mantoux test reading of ≥10 mm induration at 48–72 h and/or a positive QFTG test, were given TB prophylaxis before initiating bDMARDs. Patients were followed and monitored for features of TB and side effects of LTBI treatment.
Table 1

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Table 2

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Results: 341 patients were screened and treated with bDMARDs (Infliximab- 125, Rituximab- 87, Adalimumab- 37, Golimumab- 35, Etanercept- 23, Tocilizumab- 20, Secukinumab- 14). 51 patients (14.95%) were positive for LTBI (TB Gold Positive-35, Mantoux positive- 2 and both positive- 14 patients). They were treated with rifampicin + isoniazid for 3 months (3HR). bDMARDs were started 4 weeks after starting LTBI treatment. Incidence of TB patients in LTBI group was 1.96% (1 in 51) and 1.37% (4 in 290) in non-LTBI group. 4 patients were on Infliximab and the 5th patient received adalimumab after failure of infliximab. Only 3 patients (5.8%) developed side effects on 3HR regimen of which 1 discontinued LTBI treatment.

Conclusion: Tuberculosis can develop even in LTBI negative patients who are on TNF inhibitors and hence require regular monitoring. bDMARD other than Infliximab can be preferred to reduce TB incidence. 3HR regimen had a safer side effect profile with better treatment completion rate.


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Fever in rheumatology practice

R N Tejaswani; ESIC Medical College and Hospital, Hyderabad, Telangana, India

Background and Objectives: Fever in AIRD patients contributes to morbidity significantly and at times to mortality. Evaluation of fever is challenging in these patients due to overlapping clinical features of infection and autoimmune disease, masking of clinical features of infection in patients who are on steroids, various infections can present with clinical manifestations similar to AIRD. Clinical evaluation, serologic markers, infection workup including blood cultures and imageology will all be necessary to arrive at a diagnosis and at times may not be sufficient enough to differentiate infection from disease activity.

Materials: This is a retrospective study done in the department of Rheumatology, ESI Medical college & Hospital from April to July 2022. Fourty eight patients admitted to rheumatology department for evaluation of fever were included in this study. Data was collected from online discharge summaries, MRD case sheets and analysed using frequencies, percentages, mean+SD etc.

Results: Among the patients who were admitted for evaluation of fever CTD constituted 36 (75%), JIA-4 (9%), SPA- 3 (6%), INFECTIONS-3 (6%), VASCULITIS-2 (4%). Among CTD- RA– 14, SLE – 14, Overlap syndrome- 3, Antisynthetase syndrome- 2, Systemic sclerosis-2, Sjogrens- 1. Infections were the most common cause of fever among RA patients, m/c- Respiratory tract infection followed by UTI. Disease activity was moderate to high in 78.5% of patients who had infection simultaneously. Infections were also the common cause of fever in SLE, m/c - GI tract, UTI and skin and soft tissue infections. Disease flare was also seen in 37.5% of patient of SLE having infection simultaneously. 42.5% of SLE patients had fever due to disease flare alone - non adherence to treatment.

Conclusion: It is necessary to identify the aetiology of fever and treat accordingly in patient with AIRD as the mainstay of treatment i.e. immunosuppression acts as a double edged sword and may result in significant morbidity and mortality. Likewise, it is also prudent to educate all AIRD patients regarding the disease and need for long term compliance.


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Tropical pyomyositis in immunocompetent hosts posing diagnostic challenges: A case series

Anuj Singhal, K K Kompella, I P Dubey, Sohil Khan, Kirti Mahanan, Abhilash Singh

Background: Pyomyositis is an acute bacterial infection of skeletal muscle, which results in localized abscess formation. Diagnosis in such cases can be delayed because the affected muscle is deeply situated and local signs are not apparent which lead to diagnose it as inflammatory pyomyositis. However inflammatory pyomyositis is very rare in comparison of tropical myositis.

Case Presentation: In last one year four such interesting cases were admitted to this tertiary care hospital with different clinical presentations. In this series, the cases described were of four previously healthy immunocompetent persons who were admitted to different smaller hospitals with varied clinical presentations including muscle and joint pain with fever. All four patients were transferred to bigger hospital due to diagnostic dilemma of inflammatory myositis. After thoroughly workup with help of CT scan, MRI and PET scan they were diagnosed with pyomyositis. Therapy was started with antibiotics and subsequently changed as per culture reports. The minimum length of therapy was 3 weeks and maximum of length was 8 week for complete recovery.

Conclusions: Pyomyositis in this series was related to factors affecting the muscle with no significant strenuous exercise or direct muscle trauma. The visual assessment of FDG uptake enabled to comprehensively evaluate skeletal muscle. The initial therapy consisting of cephalosporin and teicoplanin was found effective. The minimum length of in hospital treatment was four weeks. This series reinforces that tropical pyomyositis is not an exclusive pathology of immunocompromised persons. Pyomyositis need to be kept it in differential before coming to conclusion of inflammatory myositis.


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Epidemiology, clinical features, and risk factors associated with chronicity in chikungunya arthritis: A single-center ambi-directional observational study

Pooja J Belani, Saibal Das1; Divine Life Hospital, Kutch, Gujarat, 1ICMR - Center for Ageing and Mental Health, Kolkata, West Bengal, India

Background: Chikungunya fever (CHKF) manifests as fever, rash and arthralgia/arthritis. 4.1-78.6% patients develop chronic disease (>3 months duration). Female gender, increasing age, symptoms(vomiting, dyspnea)and initial functional disability are associated with chronic arthritis in CHKF. Steroids have been used in acute viral infections previously. Antiviral role of steroids is being recognized recently. However, there is no evidence to support/refute the use of steroids in the early phase of CHKF when there are disabling symptoms in spite of supportive treatment.

Objectives: To study the epidemiology, clinical features and risk factors associated with chronic disease in confirmed cases of CHKF.

Methodology: This single-center, ambi-directional observational study included patients who presented to the outpatient department with CHKF (serology/PCR positive) during the 2021 outbreak.[1] Patients having encephalitis, myelitis and neuritis manifestations of the diseases that mandated early use of steroids were excluded. Demographic and clinical characteristics of the patients were recorded. Various risk factors of chronicity in CHKF arthritis, including the early use of steroids, were evaluated using the chi-square test and binary logistic regression.

Results: A total of 57 patients (72% females) were included, of whom 69% were of <60 years of age. Median age at presentation was 50 years (range: 28-72 years). 56.8% of patients had an acute disease. The most common manifestations were arthritis (100%), followed by fever (86%), myalgia (40%), tenosynovitis (38%), skin rash (38%), sudden-onset stiffness making patients bedridden/dependent (38.4%), backache (18%), gastrointestinal involvement (11%), enthesitis (5%) and joint deformity (9%). The most commonly involved joints were small plus large joints (88.6%) and bilateral upper and lower limbs (77%). Raised ESR (>40 mm)and raised CRP were found in 64.7% and 55.1% of the patients, respectively. Leucopenia (36.3%), thrombocytopenia (17.8%), deranged liver function (23%) and concomitant dengue infection (12.5%) were also noted. 26% patients received steroids within the initial 2 weeks of infection. The only risk factor that was associated with chronicity was sudden-onset stiffness at presentation.

Conclusion: In the studied population, CHKF was common in middle-aged females, with a majority of the patients having an acute disease. Most common manifestations were joint pain followed by fever, myalgia and skin rash. Sudden-onset stiffness at presentation, but not early steroid initiation in acute CHKF arthritis was associated with chronicity.


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Musculoskeletal manifestations of brucellosis: A case series

Sanket Shah, Shyam Shah1, Ronak Bhalodiya2; GCS Medical College, 2Zydus Hospital, Ahmedabad, Gujarat, 1CMC, Vellore, Tamil Nadu, India

Background: In an endemic area of brucellosis, a patient with fever and difficulty walking into the clinic would be regarded as having brucellosis until it was proven otherwise. Brucellosis with its spinal, articular, and entheseal involvement can mimic inflammatory arthritis especially spondyloarthritis.

Objectives and Methods: We conducted a prospective analysis of eleven patients of brucellosis with musculoskeletal manifestations seen at tertiary care center of Gujarat.

Results: A total of 11 patients were diagnosed with brucellosis during the study period; the mean age was 27 years; the majority were males (n=7). The most common risk factor for Brucella infection in the cohort was direct cattle exposure (n=8). All 11 patients had indolent fever. Musculoskeletal manifestations included peripheral arthritis in two patients, Axial involvement (Spondyolitis/Sacroilitis) in 8 patients and tenosynovitis with arthritis in one patient [Figure 1]. Diagnosis of Brucellosis was proven serologically in 8 patients and with culture in three patients. All patients were treated with antibiotics, (Doxycycline+Rifampicin+Streptomycine) along with symptomatic management. On median follow-up of 6 weeks patients, they were symptom free [Table 1].

Conclusion: In case of monoarthritis sacroiliitis or spondylodiscitis, the index of suspicion should be high in regions where the brucellosis is endemic.


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Efficacy of denosumab after 1 year of therapy in patients with osteoporosis having various autoimmune rheumatological diseases: An observational study

Shyamashis Das, Debanjali Sinha; Department of Rheumatology, Institute of Neurosciences Kolkata (I-NK), Kolkata, West Bengal, India

Background: Efficacy of denosumab in post-menopausal osteoporosis is well known. However, there is relative paucity of data of denosumab in osteoporosis patients with autoimmune rheumatological diseases (ARDs).

Objective: In this prospective observational study we wanted to evaluate the efficacy of denosumab (originator brand) in patients with osteoporosis having various ARDs.

Methods: Patients with osteoporosis, diagnosed by bone mineral density (BMD) T-score <-2.5 as per World Health Organization definition, were recruited from Rheumatology OPD of Institute of Neurosciences Kolkata, from January 2019 to June 2021 prospectively. The patients with osteoporosis who did not have any ARDs were excluded. Patients with neoplastic disorders, metabolic bone disease other than osteoporosis and hypovitaminosis D were also excluded. After recruitment, denosumab was given every 6 monthly for minimum 3 doses. All subjects received oral calcium supplement ≥500 mg and vitamin D3 ≥ 500 IU daily. BMD by DEXA were performed in all patients at baseline and after the 3rd dose of denosumab (between 12-18 month).

Results: Out of 66 recruited patients, 53 (48 females) patients completed at least 3 doses of denosumab. Underlying rheumatological diseases were as follows: rheumatoid arthritis (50.9%), connective tissue diseases (including SLE, MCTD, systemic sclerosis and Sjogren's syndrome) (13.2%), ankylosing spondylitis (7.5%), psoriatic arthritis (7.5%) and vasculitis (7.5%). Seventeen patients had previous history of fragility fracture. Baseline T-score of lumber spine, neck of femur and forearm were -3.65 (SD 0.78), -2.91 (SD 0.55) and -3.31 (SD 0.92) respectively. There were 7.5% (95% CI 5.8%-9.1%), 3.8% (95% CI 1.9%-5.6%) and 2.7% (95% CI 0.15%-5.1%) improvement of BMD at lumber spine, neck of femur and forearm (P < 0.0001, <0.0002 and <0.03 respectively). There were incidents no fractures or withdrawals due to adverse events during study period.

Conclusion: Denosumab demonstrated a significant improvement of BMD in osteoporosis patients with ARDs.


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Assessment of glucocorticoid induced osteoporosis treatment in accordance to prevailing guidelines in a tertiary care hospital

Abhijeet Kr. Agrawal, P D Rath, Swetal Chauhan, Rahul Bisaralli, Hiren Kalyani, Akanksha Kapoor; Department of Rheumatology, Max Hospital, New Delhi, India

Background: Rheumatology is a branch where use of glucocorticoids is prevalent for control of disease activity. Long term use of glucocorticoids has serious complications like glucocorticoid induced osteoporosis (GIOP). GIOP is an underplayed condition where only around 15 % patients receive optimal treatment despite of ACR 2017 guidelines for the same. Patients who fall under moderate to high risk of GIOP should be on bisphosphonates. Assessment of GIOP is done via measuring the bone strength, of which bone mineral density (BMD) is the commonest tool. However, it gives no value on qualitative defects in the bone. FRAX (fracture risk assessment tool) is a simple, cost effective, bedside, composite clinical score, that provides a 10-year fracture risk assessment for any major osteoporotic fracture and Hip fracture.

Objectives: The study aims to evaluate if patients on long term glucocorticoids assessed adequately for glucocorticoid induced osteoporosis, as per ACR GIOP guideline 2017.

Methods: All patients on prednisone dose ≥ 2.5 mg/day for a period ≥ 3 months who are under moderate to severe risk of GIOP will be evaluated in this study. FRAX score will be calculated for the same.

Results: Till now 49 patients have enrolled in this study. There are 39 females and 10 males. Average age of the participants is 49.67 years. The average dose of glucocorticoids is 8.5 mg. A total of 7 (14.28 %) patients received treatment for preventing GIOP. 9 (18.3%) patients were having moderate risk and 5 (10.42%) patients were having high risk of GIOP, none of them received bisphosphonates.
Figure 1: Left wrist flexor tenosynovitis

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Table 1: Treatment and outcome of patients of musculoskeletal brucellosis

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Conclusion: Long term intake of glucocorticoids leads to increased risk of osteoporotic fractures which is often overlooked. This study highlights this lacuna in daily clinical practice.


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Clinical characteristics of a sarcoidosis cohort in a tertiary care centre in South India

Kaamil Zubair Rabbani Amaanulla, Rukhsar Abdur Rahim Mulla, S Rajesh1, M Bhuvanesh1, Abitha Aliyar1; Departments of Internal Medicine and 1Rheumatology and Clinical Immunology, KIMSHEALTH, Thiruvananthapuram, Kerala, India

Background: Sarcoidosis is a multisystem granulomatous disorder and the management involves different subspecialists like pulmonologist, rheumatologist & ophthalmologist depending on the manifestation.

Objective: This study is aimed at describing the clinical characteristics of sarcoidosis in a Rheumatology unit of a tertiary care centre in South India.

Methods: We did a retrospective case study of patients diagnosed with sarcoidosis based on clinical, radiological and pathological evaluation, by collecting data from the electronic medical records.

Results: Our analysis revealed a female preponderance of 77.8% & 69% of cases were aged above 30 years. Thoracic involvement was the most common manifestation with 63% with 55% mediastinal adenopathy & 48% lung parenchymal involvement. This was followed by musculoskeletal involvement with 37.5%. Cutaneous and ocular involvement were almost similar with 29.6% & 24.7% respectively. Granulomatous hepatitis was observed with 6.2% and splenomegaly in 12.3%. Only 8.6% of patients had cardiac and CNS involvement.

Conclusion: In our study, we noted that there was a female preponderance in the diagnosis of sarcoidosis with a predominant thoracic involvement similar to Ungprasert P et al. The heterogenous manifestations of sarcoidosis has also got an ethnic variation and overall involvement of organs need to be looked at prior to definite management of these cases.
Table 1

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Burden of immunological patients at medanta superspeciality hospital Indore – A five years audit

Vivek Kattel, Neetu Gupta1, Virend Tilwee, Sanjay Geed, Sourabh Malviya; Department of Rheumatology and Clinical Immunology, Medanta Superspeciality Hospital, 1Department of Anaesthesiology, MGMMC and MY Hospital, Indore, Madhya Pradesh, India

Background: Medanta Superspeciality hospital Indore, started rheumatology services since December 2014 providing services to urban, peri urban and rural areas of Madhya Pradesh and neighbouring states.

Objectives and Methods: Clinical Audit was done for 5 years from December 2014 to November 2019.

Results: By the end of five-year 10,441 new rheumatology patients visited the hospital with median age of 47 years (range 4 years to 98 years) [Table 1]. The most common cases associated with predominantly arthritis were of rheumatoid arthritis (36.8%), followed by Spondyloarthritis (14.7%), osteoarthritis (11.6%), Post Chikungunya arthritis (3.1%). Among connective tissue disorder the common disease was Systemic Lupus Erythematosus (2.8%), undifferentiated connective tissue diseases (1.8%), Scleroderma (0.74%) vasculitis (0.65%) and mixed connective tissue diseases (0.3%) [Table 2]. Among 10441 cases 12.87% (1344) went under intraarticular injections. The most common site was knee (96.6%). The etiology was inflammatory arthritis (65%) verses osteoarthritis (35%). Among 869 cases of inflammatory arthritis receiving intraarticular Rheumatoid arthritis, Spondylarthritis and Undifferentiated inflammatory arthritis was 67%, 17% and 5% respectively. Mean VAS score pre injection, post injection at 30 minutes, 4 weeks and 12 weeks were 96 (±9), 19 (±4), 68 (±14) and 80 (±15) respectively with statistically significance. The proportion of immediate adverse event was 5.5% without any life/joint-threatening events like anaphylaxis and septic arthritis. Among 10,441 patients in hospital admission was 3.6% (377). The most common causes of admission were disease flare (261, 69%) due to delay in primary diagnosis (107), partial treatment (32) or poor compliances (122) followed by infections (97, 26%) mostly non tubercular (94). The average hospital stay was 3.5 days with mortality of 0.27%. The average follows up of individual patient was 4.2 visits per annum.

Conclusion: This is a single centre study presenting the burden of disease in a Rheumatology clinic. Most common disease was Rheumatoid arthritis followed by Spondyloarthritis.


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To study the prevalence of various ocular manifestation in rheumatological diseases in tertiary care center of Central and North India

Mahendra Pratap Singh, Anupriya Mehrotra1, Ravi Mehrotra2, Mukesh Mourya3; ABVGMC, 1ASG Eye Hospital, Vidisha, 2PCMS and RC, Bhopal, Madhya Pradesh, 3Department of Rheumatology and Clinical Immunology, KGMC, Lucknow, Uttar Pradesh, India

Background: Rheumatologist consider eye as a microcosm of the body, it's complex structure frequently reflects inflammation elsewhere. In many rheumatic patients Ocular manifestation such as Dry eye, uveitis, Episcleritis, Scleritis etc could be a presentation before rheumatic disease are manifested. Eye could be a sign of exacerbation of many rheumatic diseases.

Objectives: To study the prevalence of various ocular manifestation in rheumatological diseases.

Method: 200 patients with various rheumatological diseases of age group 5-60 years were selected from Rheumatology clinic in PCMS&RC (M.P), ABVGMC Vidisha (M.P), KGMC Lucknow (U.P) during the period of one year April 2021 to April 2022, detailed clinical history,physical examination, ophthalmological examination using Direct and indirect Ophthalmoscope, slit lamp examination and dry eye work up was done. Various blood and radiological investigations and diagnostic criteria were used during the study.

Results: (1) Among 200 rheumatic patients 112 Had RA out of which 31% had ocular manifestation, KCS being most common in 60%. 42 had AS, having ocular involvement in 30.9% all having Anterior uveitis. 26 had SLE, having ocular involvement in 30.7 %, KCS being commonest in 75%. 11 patient had Sjogren syndrome out of which 72%having ocular involvement and all having KCS. 5 had Psoriatic Arthritis out of which 20% having ocular involvement as Anterior uveitis.4 patients had JIA with 50% having ocular involvement as Anterior uveitis. (2)Out of 200 rheumatic patients 67 had ocular manifestations with maximum prevalence of KCS (17.5%) followed by Anterior uveitis (11.5%).

Conclusion: (1) Ophthalmologist can help in early detection of Rheumatological diseases, there Collaborative work with Rheumatologist can delay or prevent many irreversible ocular complications of rheumatological diseases. (2) ANA Positivity increases the probability of having ocular manifestation among rheumatic diseases patient.


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Cross cultural adaptation and validation of Hindi and Marathi versions of musculoskeletal health questionnaire score

Priyadarshi Prajjwal, Raunak Ranjan, Sakshi Manglik, Shraddha Nakum, Rajvardhan Singh, Anish Lodha, Ayush Kumar, Anjali Shukla, Deepti Agarwal, Sandeep Kansurkar, Jitendra Oswal, Kavita Krishna; Department of Clinical Immunology and Rheumatology, Bharati Vidyapeeth (Deemed To Be University) Medical College and Hospital, Pune, Maharashtra, India

Background: Composite scores have been designed to measure several aspects of musculoskeletal health. MSK-HQ is one of such recent scores which has been validated to evaluate these aspects of health in musculoskeletal pathways. The score is originally published in English language, and it has been validated in several European languages. It has not been tested in Indian settings. We have translated and adapted MSK-HQ score to local languages Hindi and Marathi as per International Society for Pharmacoeconomics and Outcomes Research (ISPOR) guidelines with permission. If validated, this can be valuable tool for assessment of musculoskeletal health of Indian patients.

Objectives: To evaluate different measures of musculoskeletal health in hospital outpatient department as per MSK- HQ questionnaire. To assess it for internal consistency, reliability and compare it with other existing health measures like EQ-5D-5L VAS.
Table 1: Yearly distribution of the patients

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Table 2: Common diagnosis of rheumatology disorders

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Methods: Consecutive patients attending outpatient clinic with musculoskeletal complaints at Rheumatology, Orthopaedics, Spine Surgery and Physiotherapy departments were enrolled. A total of 302 Participants filled up MSK HQ and EQ-5D-5L questionnaires in Hindi or Marathi language. Out of which 290 were considered for final analysis after excluding incomplete entries.

Results: Musculoskeletal complaints have widespread impact on other parameters of health [Figure 1]. The score showed good internal consistency, Crohnbach's alpha (0.93) and reliability in test-retest (interclass coefficient of 0.94). Construct validity was demonstrated by the good correlation between the respective versions of MSK-HQ and EQ-5D-5L VAS for health (p<0.001, rho = 0.6) [Figure 2].

Conclusions: Our study shows that MSK HQ score when translated and adapted to Indian languages can prove as an useful tool for evaluation of musculoskeletal health among the population


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Undergraduate rheumatology in India: Time to be fair

Deepti Agarwal, Sandeep Kansurkar, S Srilakshmi, Kavita Krishna; Department of Clinical Immunology and Rheumatology, Bharati Vidyapeeth (Deemed To Be University) Medical College and Hospital, Pune, Maharashtra, India

Background: Musculoskeletal diseases are one of the major causes of morbidity in community and an MBBS graduate who is a “First contact Physician” in the community needs to be adequately trained to recognise these. Rheumatology as a subspeciality of medicine has lesser teaching hours dedicated to it as opposed to other specialities like cardiology or neurology and hence the undergraduate medicine students are bound to become inadequately trained.

Objective: To evaluate the awareness and knowledge about rheumatological illness among final year medical undergraduates and interns.

Methodology: This is a questionnaire based cross sectional, study. The questionnaire consisted of 10 questions in various domains which were framed to assess the level of exposure and to rheumatological illness and knowledge gained during undergraduate training. The questionnaire was circulated in both print and digital form (as google forms) to multiple colleges form different regions of India.

Results: A total of 430 final year MBBS students and interns completed the survey. According to them, Rheumatoid arthritis (89.4%) and osteoarthritis (75.8%) were amongst the most frequently encountered rheumatological conditions. 75% students opined that examination of knee joint was taught and other joints like ankle (33%) and small joints of foot (27%) received the least attention [Figure 1]. According to 62.6% students, the most difficult aspect in learning rheumatology was inadequate training in musculoskeletal examination. When tested about their rheumatological knowledge, most fared poor (for example 51.4% students were of the opinion that Osteoarthritis can be treated with DMARDs, 64.9% students felt that methotrexate is a dangerous drug when given with frequent monitoring). We also found that most of the students were under confident when it came to evaluating a rheumatological complaint (eg: students gave a confidence level score of 5 out of 10 when it came to differentiating between pleural effusion and consolidation, whereas the average score was 3.2 out of 10 to differentiate between inflammatory vs non inflammatory arthritis).

Conclusion: Our study throws light on the inadequate nature of rheumatology training for MBBS graduates in the existing system and highlights the need for implementing better training.


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Clinical feature and treatment response of IgG4-related disease managed at a tertiary care centre

Sandeep Yadav, Girija Sachdev, C Balakrishnan; PD Hinduja National Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Background: IgG4-RD is a rare fibro-inflammatory disorder with protean manifestations.

Objectives: We present clinical features, laboratory features, radiology, histology and treatment outcome of IgG4-RD managed at our centre.

Methods: Diagnosis of IgG4-RD was made based on 2020 revised comprehensive diagnostic criteria. Treatment responses were determined using IgG4 responder index.

Results: Forty patients (males 25) were analysed with mean age of 45.18 (15.8). Disease classification was: Definite -24 (60%), possible-11 (27.5%), and probable- 5 (12.5%). Single organ involvement was seen 30/40 (75%) and multisystem involvement in 10/40 (25%). Paranasal sinuses (35%), pulmonary parenchymal involvement (30%) and retroperitoneal (RP) tissues (30%) were commonly involved. RP involvement was often combined with periaortic involvement (27.5%) and urethral obstruction (17.5%). Serum IgG4 levels were normal in only 3/ 40 patients, with median serum IgG4 levels of 5.65 g/l (IQR 3.05-11.80 g/l). Biopsy (40/40) showed fibrosis, and lymphoplasmacytic infiltrate in all cases, with storiform fibrosis in 3 (7.5%) and obliterating phlebitis in 4 (10%) patients. All patients initially received steroid therapy 0.5-1 mg/kg/day taper. Other immunosuppression used were Tamoxifen 5 (12.5%), Methotrexate-19 (47.5%), MMF-11 (27.5%), Rituximab-5 patients (12.5%) and Azathioprine-2 (5%). At 1 year, IgG normalized in 13/26 (50%); and in 50% it had improved. No patient had complete resolution on repeat imaging. There was improvement or stabilization in 84% of patients and worsening in 16 %. At 1 year IgG4 responder index improved or stabilized in 80 % of patients and worsened in 20 %. In patients with at least 18 months of follow-up, the disease had a relapsing course in 13/16.
Figure 1: Average score of parameters of MSK- HQ (score 0 to 5) among the participants. Musculoskeletal complaints have widespread impact on other parameters of health

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Figure 2: MSK HQ score has good correlation (p<0.001, r = 0.6) with state of health as measured by visual analogue scale in EQ-5D-5L score

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Conclusions: In this series of biopsy proven IgG4-RD, upper airways, lower airways and retroperitoneal area were commonly involved. Typical histopathology of storiform fibrosis and phlebitis were uncommon. Response to treatment was good, but the relapsing nature of the disease was seen in a significant proportion of patients.


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A study of interstitial pneumonia with autoimmune features in patients of interstitial lung diseases of unknown origin

Gunjan Lalwani, Ajay Bhatta, M B Indu, Desh Deepak1, Mala Chhabra2, Brijesh Sharma, Nandini Duggal2; Departments of Medicine, 1Respiratory Medicine and 2Microbiology, ABVIMS and Dr. RML Hospital, New Delhi, India

Background: Many patients with Interstitial Lung Diseases (ILD) of unknown origin have clinical features and antibodies suggesting presence of an autoimmune pathology but they do not fulfill the defined criteria of any connective tissue disorder. In 2015, a criterion with clinical, morphological and serological domains was established by American Thoracic Society/European Respiratory Society (ATS/ERS) task force to classify such patients into a distinct entity called Interstitial Pneumonia with autoimmune features (IPAF).

Objectives: To study the prevalence of IPAF in patients with ILD of unknown origin and to study the clinical, morphological and serological features in IPAF patients.

Methods: This cross-sectional observational study conducted between 1st January, 2021 and 31st May, 2022 in a tertiary care hospital in New Delhi enrolled 80 patients with ILD who did not fulfill the criteria for any connective tissue disorder or other known causes of ILD. Clinical profile and the HRCT pattern of the enrolled patients were studied and serum autoantibody testing was done. Patients who fulfilled the ATS/ERS criteria were re-classified into IPAF.

Results: 61.25% patients with ILD of unknown etiology fulfilled the criteria for IPAF. 77.5% of IPAF patients were females, with the mean age being 50.69±12.88 years. The most common HRCT pattern in IPAF was NSIP (85.71%). 51.2% IPAF patients showed features from clinical domain, most common being Raynaud's phenomenon (28.57%) and inflammatory poly arthritis or polyarticular joint stiffness (24.48%). Autoantibodies were seen in all IPAF patients, most common antibody being RF (69.38%) followed by ANA (30.61%) and antiSSA (20.40%).

Conclusion: A large number of patients with ILD who did not fulfill any criteria for connective tissue disorders or any other known causes of ILD were categorized as IPAF based on antibodies, clinical and radiological features. Further, long term studies with larger sample size are needed to study the disease course and management in such patients.
Figure 1: Most commonly encountered problems in learning rheumatology

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Profile of patients with ANA and anti U1RNP positivity and not fulfilling any classification criteria for definite CTDs

R Nagendran, P S Arulrajamurugan, R Ramesh, S Mythili; Department of Clinical Immunology and Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Classification criteria for UCTD have not been defined. However, Mosca et al. proposed preliminary classification criteria, comprising (1) signs and symptoms of CTD, but not fulfilling the criteria for any defined CTDs, (2) presence of antinuclear antibodies determined on two different occasions. Currently, there is also no methods to establish the chances of progression to other CTDs and prognosis.

Objectives: To study the profile of patients with ANA and Anti U1RNP positivity and not fulfilling any Classification criteria for definite CTDs in a tertiary care center.

Methods: This is a retrospective review of case records of patients not fulfilling any Classification criteria for definite CTDs who attended IOR during the past 1 year. Patients fulfilling Classification criteria for CTDs, Children less than 16 years, Active malignancy, Tuberculosis were excluded. Analysis of clinical profile, lab and antibody profile were done.

Results: 22 patients were included, with a mean age at diagnosis and disease duration of 31.9±13.9 and 1.5±3.5 years, respectively. Most patients were female (86.3%). The major manifestations include arthralgia/arthritis (36.6%/27.2%), Raynaud's phenomenon (36.6%), Skin rashes (22.7%), Dyspnea (22.7%), and Neurological Manifestations (18.1%). Other manifestations include Fever (13.6%), Myositis (9.09%), Skin thickening (9.09%), Gangrene (9.09%), Sicca (4.54%), GERD (4.54%). Laboratory profile shown Anemia of chronic disease (68.1%) as the major hematological abnormality with 1 patient showing Aplastic anemia. Leucopenia (9.09%), and thrombocytopenia (4.54%), was comparatively less. Imaging showed ILD- NSIP pattern (22.7%), and UIP (4.54%), Severe Pulmonary hypertension (13.6%). Immunological profile showed Anti U1RNP- 100% (inclusion criteria), Anti-Ro60 -18.1%, Anti-Ro52- 18.1%, Anti-Pm-Scl- 9.09%, Anti-Scl70, and Ku- 4.54%.

Conclusion: Arthralgia/ Arthritis, Raynaud's phenomenon, skin manifestations and anemia were the most common. 2 patients were labelled as early SLE, 1 as UCTD, 15 as Early UCTD and 4 Patients have no CTD diagnosis. Further follow up is needed for definitive diagnosis.


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Evaluation of awareness regarding rheumatic diseases in the general population of Kerala, India

D Marwaha, N Ajithkumar, Gattu Anuraag1, V Marwaha; Department of Commerce and Management, Amrita School of Arts and Sciences, Amrita Vishwa Vidyapeetham, 1Amrita School of Medicine, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

Background: Existing in many forms, Rheumatic Diseases (RD) are chronic autoimmune conditions that are inflammatory in nature with various physical and social consequences that cause pain, disability, and even premature death. General awareness about RD will help in better management of disease due to early diagnosis and timely intervention.

Objectives: To evaluate the awareness regarding rheumatic diseases in the general population of Kerala by surveying the visitors of a tertiary care hospital in Kochi, Kerala.
Table 1: Treatment response of patients with IgG4

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Methods: A cross-sectional study was conducted by distributing a standardized questionnaire among the visitors of a tertiary care hospital in Kochi, Kerala. 15 statements gauging general beliefs regarding myths and facts about rheumatic diseases (true/false) were given, with one point given for each correct answer. Those with a medical background (Doctors, nurses, allied health science) were excluded from the study.

Results: A total of 189 participants were included, 35% females and 65% males. The mean total score of 10.71±2.14 indicated a moderately aware population. The mean score was higher in females (p<0.01) and in participants with higher education (p<0.01). In multivariate analysis, gender (p<0.05) and education level (p<0.01) were associated with higher scores in statements related to myths. Age had no significant association with scores. Overall, the questions most incorrectly answered were about movement restrictions in rheumatic patients (46.3%) and the availability of joint replacement facilities (39.5%).

Conclusion: Awareness of rheumatic diseases in the general population is moderately good with females faring better than males. Education affects awareness positively, signaling a more targeted approach towards the lesser educated. Higher misconceptions about RD will influence healthcare-seeking behavior negatively and must be combatted through mass media efforts. Basic information must be spread comprehensively to the grassroots level for better awareness.


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Rheumatology patients fail to adhere the unknown know

Vivek Kattel, Neetu Gupta1, Sourabh Malviya; Departments of Rheumatology and Clinical Immunology and 1Anaesthesiology, Medanta Superspeciality Hospital, Indore, Madhya Pradesh, India





Background: Autoimmune disease is alarming in developing world reason being high prevalence and inadequate point of care compared to the burden. Early diagnosis and adherence to therapy is key to success against rheumatologic disorders which still remains a current challenge in Indian subcontinent. The objective of the study was to explore the reason of poor adherence among common rheumatology patients.

Methods: It was a cross sectional study carried out among 323 patients of common rheumatological disorders (Rheumatoid Arthritis (RA), axial spondyloarthropathy (axSpA), Primary Sjogrens' Syndrome (PSS) and Sytemic Lupus Erythematosis (SLE)) who presented first time in our clinic. A base line data on epidemiology, treatment and other factors associated with non-adherence was recorded using questionnaire.

Results: Among 323 enrolled patients 73% were non-complaint with previous treatment. Among 236 cases the most common reason stated by these patients were false belief that allopathic medication only controls symptoms (74%), poor control of the symptoms with previous treatment (53%), prolong duration of treatment (48%), treating physician did not counsel about disease (47%), inadequate physical examination (47%), polypharmacy (46%), inadequate time given for consultation (33%) and drugs side effects (29%) [Figure 1]. Nearly one third of patient stated reason for poor compliance as either financial constrain, lack of social support and inaccessibility. The median duration of diagnosis of the specific autoimmune diseases from symptomatic onset for RA, SpA, SLE and PSS were 3.5 years, 2.5 years, 3 years and 4 years respectively. Good compliance was strongly associated with knowledge regarding consequences of poor treatment, early diagnosis (within 1 year of symptom onset) and treatment under rheumatologist with odds ratio of 7.5, 4.1, and 3.5 respectively [Table 1].
Figure 1

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Conclusion: Delay in diagnosis, inadequate control of symptoms despite treatment, lack of knowledge regarding importance of compliance and myths about DMARDs as symptoms relieving pills were gaps in poor adherence.


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Gender equity in the Asia pacific: Comparison of Indian and Philippines rheumatology conferences

M M Kavitha, Annamalai Sowndhariya1, Vinod Ravindran2, Lisa S Traboco3, Geraldine Zamora-Abrahan3, Sheila Marie Reyes4, Pavel V Ovseiko5, Latika Gupta6; Saveetha Medical College Hospital, Chennai, Tamil Nadu, 2Centre for Rheumatology, Kozhikode, Kerala, India, 1Aster DM Health Care, Dubai, 3St Luke's Medical Center - Bonifacio Global City, Taguig, 4St Luke's Medical Center - Quezon City, Quezon City, Philippines, 5Department of Medicine, University of Oxford, John Radcliffe Hospital, Oxford, 6Royal Wolverhampton Trust, Wolverhampton, UK

Background: Equitable gender representation at conferences is essential to further progress in academia. India and the Philippines are both low and middle-income countries in the Asia Pacific, albeit with divergent gender norms and social frameworks. While India has a patriarchal gender norm, the Philippines has been said to have a relatively egalitarian gender norm.

Objectives: Our objective was to obtain an overview of gender equity at the annual conferences of the Philippine Rheumatology Association (PRA) and compare it with the Indian Rheumatology Association's annual conferences (IRACON). Methods: Using the available published PRA conference materials from 2009 to 2021, we determined the proportion of women faculty (speaker or chairperson roles). Gender was identified based on information from the organizers, online science directory networks, and a name-to-gender inference platform, Gender application program interface (API). Results were compared with published data from IRACON.
Table 1: Clinical and immunological characteristics

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Results: The overall proportion of combined PRA female faculty was 47.7% while female speakers were underrepresented at IRACON at 18%. Women were also more likely to be abstract first authors at the PRA (68%) but underrepresented at IRACON (27%). There are more females among new inductees in PRA with an M: F ratio of 1:3, while the trends are improving in India, with a notable narrowing of the gender gap among new inductees recorded (from 5:1 to 2.7:1) in the 2010 and 2015 quinquennium. The trends converged when international female faculty were analyzed at both conferences, with low representation uniformly (16% PRA, 15% IRACON).

Conclusion: We found a better gender parity at the PRA conferences, in comparison to IRACON's. Contrastingly, the gender gap was wide among international speakers in both countries. These observations suggest that cultural and social constructs may have an important role to play, and further research on other Asia Pacific countries' gender distribution in academia is recommended.


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Immunosuppression in connective tissue disease-associated pulmonary hypertension – A retrospective study

B Sasikala, Ramya Janardhana, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, Karnataka, India

Background: Data on response to immunosuppression (IS) in Connective tissue disease-associated pulmonary hypertension (CTD-aPH) is limited.

Objectives: To assess short-term outcomes of IS therapy in CTD-aPH patients, mortality and its determinants.

Methods: Charts of CTD-aPH patients who received IS between 2018-2022 were retrospectively reviewed. We have included all CTD patients with pulmonary hypertension except systemic sclerosis aPH patients with at least 2 recorded PASP and NYHA values at 6 apart after starting IS. Diagnosis of PH was based on PASP (Pulmonary Arterial Systolic pressure) of > 40 mm/hg, as determined by transthoracic echocardiography (TTE). Response to IS was defined as a change in PASP of at least 15 mm/hg and improvement in NYHA (New York Heart Association) class at 6 months of follow-up after initiation of treatment. Inability to achieve a predefined definition of response, admission due to worsening shortness of breath or death, were termed as lack of response to IS.

Result: We included 25 consecutive patients, demographic and baseline characteristics depicted in [Table 1]. Almost half [14 (56%)] of our patients at presentation were symptomatic with class 3 NYHA and had severe PH [14 (56%)] and right ventricular (RV) dysfunction in 22 (88%) on TTE. Around 2/3rd of the patient's PH was the prime indication for IS 18 (72%). Majority of our patients received high-dose steroids 18 (72%), Concomitant pulse cyclophosphamide 17 (68%) or MMF (Mycophenolate mofetil) 5 (20%). All patients received vasodilators. Treatment goal [Table 2] mean reduction in NYHA and PASP reduction > 15 mm/hg was achieved in 18 (72%) and remained unchanged in 4 (16%). Two patients expired (8%), one had a relapse of PH with severe PH and class 3 NYHA due to treatment default, and another newly diagnosed patient with severe PH, with right heart failure.

Conclusion: Immunosuppression in combination with vasodilator therapy results in good short-term outcomes in CTD-aPH patients.


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Telemedicine in rheumatology from Indian patient's perspective: Experiences, acceptability and perceived quality of care

Amitesh Khare, Vijay Laxmi1; AIIMS, New Delhi, 1GSVM Medical College, Kanpur, Uttar Pradesh, India

Background: Over the last many years, there have been rapid advancement in the field of rheumatology which has led to introduction of successful treatment of once debilitating and life-threatening conditions. Still, many challenges remain in providing rheumatology care access in India. The pandemic-initiated extensive use of telemedicine has highlighted the need for detailed research ahead of more permanent digital service delivery.

Objectives: Our study aimed to evaluate views and experiences of patients receiving telemedicine care.

Methods: Patients over the age of 18 years who received a telemedicine consult and have been suffering from a rheumatological disease were included in the study. The validated questionnaire consisted of semi structured questions and ensured that quantitative opinion on comparison of telemedicine with face-to-face consult and qualitative responses about telemedicine views and experiences was captured.

Results: Majority of the patients surveyed considered telemedicine more convenient than face-to-face for initial visits, citing easy appointments, no travelling, and reduced waiting times, as the main advantages. Challenges highlighted included poor coordination of voice calls, digital literacy issues and follow up.
Figure 1

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Table 1: Compliances proportion between rheumatologist versus other physician

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Conclusion: The rapid increase of teleconsultation was fuelled due to the pandemic. Even though the concerns highlighted in the study involve multiple stakeholders, still there are implications for a future telemedicine policy in India and it highlights the need for ongoing assessment of utilizing emerging technologies.


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The effect of ixekizumab versus adalimumab on individual components of the ACR composite score, with and without concomitant methotrexate or other conventional synthetic DMARDs at 52 weeks in patients with psoriatic arthritis

K B Rakesh, M Elaine Husni1, Sona Kamat2, Keri Brooke Stenger3, Rebecca Bolce3, Thorsten Holzkaemper3, Cameron C Helt3, So Young Park3, Jeffrey R Lisse3, Luca Idolazzi4; Eli Lilly and Company (India) Pvt. Ltd., Gurugram, Haryana, India, 1Cleveland Clinic, Cleveland, Ohio, 2St Louis University, St. Louis, Missouri, 3Eli Lilly and Company, Indianapolis, Indiana, 5University of Verona, Verona, Italy

Background: This analysis describes the effect of ixekizumab (IXE) and adalimumab (ADA) on individual ACR components at week (Wk)52, with and without concomitant methotrexate (MTX) and csDMARDs.

Methods: Patients from SPIRIT-H2H (NCT03151551, 52Wk randomized multicenter study) who met Classification Criteria for Psoriatic Arthritis (CASPAR) (N=566), were randomized 1:1 to IXE or ADA. Patients had active psoriatic arthritis (PsA), psoriasis (PsO), and were bDMARD-naïve with inadequate response (IR) to csDMARDs. Patient's Global Assessment (PtGA) and Physicians Global Assessment (PGA), Health Assessment Questionnaire-Disability Index (HAQ-DI), and joint pain were assessed by visual analog scale, and TJC and SJC as well as C-reactive protein (CRP) were analyzed. Nine patients with active PsO and BSA?3% were assessed as Psoriasis Area and Severity Index (PASI)=0 at baseline, a medical inconsistency that was resolved using medical judgment. These patients were considered PASI100 responders if PASI=0 and BSA=0 at post baseline visits.

Results: Overall, 566 patients received either IXE (N=283) or ADA (N=283). Baseline values for individual ACR components were balanced between IXE and ADA. At Wk52, IXE demonstrated efficacy across individual ACR components in the ITT population, specifically in PGA, PtGA and Joint Pain. Improvements from baseline for IXE were observed across ACR components, with or without MTX or csDMARD. The effect of MTX (with or without) was notably different between IXE and ADA in TJC68, PGA, Joint Pain, and PtGA.

Conclusion: In this analysis, there was improvement with IXE in all components of the ACR composite score at Wk52, irrespective of MTX or csDMARD use (with or without). In the ITT population, IXE showed comparable efficacy to ADA at Wk52 in all components of ACR, demonstrating improvement across musculoskeletal domains. These results may provide further confidence of the clinical benefits of ixekizumab across musculoskeletal domains in patients with PsA, regardless of MTX or csDMARD use.


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Idiopathic granulomatous mastitis – Experience from a single centre

Ridhima Ramratan Jakhotia, Deepali Gonjari1, Raman Gaikwad2, Pravin Patil3; Department of Medicine, Dr DY Patil Medical College, 1Aditya Birla Memorial Hospital, 2Sahyadri Hospital, Deccan, 3Pune Rheumatology Center, Pune, Maharashtra, India

Background: Idiopathic granulomatous mastitis (IGM) is a under recognised benign condition that presents as painful nodules of the breast. The main differentials include tuberculosis and breast cancer.

Objectives: To describe the clinical profiles and treatment outcomes of IGM.

Methods: A retrospective study of EMR from Jan 2015 to Aug 2022 was performed. A total of 26 patients were identified between the age group of 24 and 59 years (median age 34.9 ± 8.25). All had confirmed diagnosis of IGM on histology. Infections and malignancy were excluded in all patients. The average followed up duration was 24 months (6-42 months).

Results: Out of 26, 10 patients (38 %) were nulliparous. Among 26 patients, 18 were treated with a combination of methotrexate 15 mg and oral glucocorticoids (GCs), 2 were given azathioprine and GCs, 4 were prescribed GCs only and 2 patients did not start treatment. On follow up, 6 are in drug free remission, 6 are stable with ongoing treatment, 1 has recurrent relapses and 13 are lost to follow up. It was observed that 2 patients had RA, 1 Sjogren's syndrome, 1 SLE and 1 Ankylosing spondylitis.

Conclusion: IGM can be seen in nulliparous women. Majority of patients responded well to treatment with conventional DMARDs like methotrexate and GCs. Nearly 20% patients have associated auto-immune diseases.


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To evaluate the baseline functional and radiological characteristics of CTD-ILD patients (A prospective, cross-sectional study)

Itishree Singh, Puneet Saxena; Army Hospital Research and Referral, New Delhi, India

Background: The most common pulmonary manifestation found among patients of connective tissue diseases (CTD) is Interstitial lung diseases (ILD). Several studies have been able to focus on specific CTD-ILD groups such as Systemic Sclerosis and Rheumatoid Arthritis where the authors have found a correlation between radiological scores and the functional capacity of the patients. Hence this study was conducted to analyse the functional-radiological characteristics of CTD-ILD patients and their sub-groups in the Indian cohort.
Table 1: Demographic and baseline characteristics

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Table 2: Outcome and response after 6 months of immunosuppressive therapy

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Objectives: The aim of the present study was to identify baseline radiological characteristics and functional characteristics among CTD-ILD patients.

Methods: The study was conducted between March 2022 and July 2022 at the Pulmonology Department in AHRR, a tertiary care centre. Patients diagnosed with any rheumatological disorder that involved lung manifestations of ILD were enrolled in our study. The different rheumatological disorders included were: Rheumatoid Arthritis (RA), Primary Sjogren's Syndrome (PSS), Systemic Sclerosis (SSc), Mixed Connective Tissue Disorder (MCTD), Undifferentiated connective tissue disease (UCTD), Polymyositis/Dermatomyositis. The investigations (immunological, radiological, functional) were performed by routine clinical practice and were prospectively retrieved. Patients who were unwilling to participate, had severe comorbidity or were unable to perform all the required tests were excluded.

Results: The most common presentation found on HRCT of each CTD-ILD subgroup was interlobular septal thickening and intralobular interstitial thickening (80%), followed by ground-glass opacities (48.8%), honeycombing, microcysts, macrocysts, traction bronchiectasis and nodules were also observed. The most common pattern of CTD-ILD was nonspecific interstitial pneumonia (NSIP) (48.8%) followed by UIP (31%). Most of the population among the patients enrolled were females (87%). Spearman's correlation conducted showed a moderate correlation (r=0.506) between 6-minute walk distance and Forced Vital Capacity among CTD ILD patients.

Conclusion: The most common radiological pattern found in CTD-ILD patients was NSIP followed by UIP with a positive correlation in spirometry and 6MWT.


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Granulomatous mastitis: Report of a series

Sapan C Pandya, Taral Parikh1, R Solanki; Vedanta Institute of Medical Sciences, 1Zydus Hospital, Ahmedabad, Gujarat, India

Background: There is scarce data on this entity from our country. Many of these are unnecessarily subjected to mastectomy which can be a psychological trauma to the subject. So we decided to study demography, clinical features and outcome in our patients.

Objective: Descriptive study of cases of idiopathic GM reported to our OPD with outcome.

Methods: Retrospective analysis of cases collected from January 2004 to August 2022 was done. The diagnosis was based on biopsy. Infections had been ruled out by culture of tissue in all cases – they were referred only after that. Descriptive statistics were applied and follow up data, where available (telephonically) was analysed. Informed consent was taken.

Results: Telephonic/OPD follow up done in August 2022: Could not connect - 5, 1 had died out of 16; 7 were in remission, had taken treatment (average) for a year; 6 patients took only for 2 months and are still getting relapses; 2 presented recently and are on follow up; 1 patient is on cyclophosphamide (keratitis), another on mycophenolate mofetil + methotrexate (EN); 1 underwent surgery after 2 months and is in remission.

Conclusion: Idiopathic granulomatous mastitis cases present to rheumatology OPDs and after ruling out infections and malignancy, can be managed with immunosuppressives with most patients doing well and only a few needing surgery.


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Audit of point of care ultrasound in rheumatology – A single centre experience

Vikramraj K Jain, V A Deepika Ponnuru, Sharath Kumar; Optima Superspecialty Rheumatology Hospital, Bengaluru, Karnataka, India

Background: Point of care ultrasound (POCUS) has become an important tool in rheumatology practice for diagnostic, monitoring and intervention purposes.

Objectives: We wanted to audit the current use of musculoskeletal ultrasound in our practice especially the indications, underlying diseases, type and number of joints and the impact of ultrasound on patient management.

Methods: This was a retrospective study using electronic medical records of all patients from Feb 2022 to Aug 2022. All patients who underwent POCUS and with complete data were included in the study. Ultrasound was done by 2 rheumatology consultants at our centre with previous ultrasound training.

Results: Of the 2875 patients who underwent in-person rheumatology consultation at our centre between February to August 2022, 129 patients (4.5%) who underwent POCUS and having complete medical records were included. Nearly 73% were female (94/129) with a median age of 52 years. Majority of scans were for diagnostic indication (81.4%) while 5.4% scans were for guided injections. Average of 3.03 joints were scanned for each patient with a maximum of 24 joints screened in a single patient. Nearly 53.1% (68/128) patients were scanned for multiple joints. Three-fourth (75%) scans were abnormal. The most common pre-ultrasound diagnosis was rheumatoid arthritis (45/129) followed by osteoarthritis (13/129), psoriatic arthritis (7/129), spondyloarthritis (6/129) and gout (4/129). The most common large joint scanned was knee and the most common small joint scanned was finger joints. In more than half the patients (54.3%) the diagnosis changed after POCUS. Treatment plan changed in 57.3% (71/124) patients.

Conclusion: POCUS was done in 4.5% patients who came for rheumatology consultation. Majority were middle aged females. Most common indication for ultrasound was diagnostic. Rheumatoid arthritis was the most common underlying disease. Management plan changed in nearly 60% of patients after ultrasound.


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The Yin and Yang of immune checkpoint inhibition: Retrospective study on risk factors for severe immune related adverse events

Sumanth Madan, C B Mithun, K Pavithran1, G Raveena1, D R Spoorthy Raj, Bhagyasree Venkat2, Sekhar V Easwar, Niveditha Kartha3; Departments of Clinical Immunology and Rheumatology, 1Medical Oncology and 2Amrita Medical College, 3Department of Biostatistics, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Immune checkpoint inhibitors have rapidly grown to represent pillars of oncological therapeutics. As accessibility to ICIs continue to increase, collateral immune related adverse events and onus of therapy lies in the liaison with rheumatologist and oncologist. Most IRAEs are mild and do not warrant stoppage of therapy. Here we explore the clinical profile and risk factors to develop IRAEs.

Objectives: To study the clinical characteristics and laboratory profile of patients receiving immune checkpoint inhibition and risk factors to develop IRAE.

Methods: This is a retrospective study including all malignancies treated with ICIs from June 2019 to June 2022 at a tertiary hospital. Demographic, clinical profile and laboratory reports of all patients receiving ICIs were collected from the electronic medical records. Patients receiving different ICIs were included in the study. Patients with insufficient laboratory data were excluded from study. IRAEs were documented as per treating oncologist, dermatologist, and rheumatologist. The severity was graded according to common terminology criteria for adverse events (CTCAE). The factors determining development and severity of IRAEs were studied.

Results: A total of 76 patients received ICIs, of which 45 were included in the study. The mean age of the study population was 60 years. Majority of patients suffered from metastatic disease – head and neck carcinomas being most common. Pembrolizumab was the most common ICI used followed by nivolumab. A total of 18 (40%) patients developed IRAE, with majority being mild (66%). The most common IRAEs were cutaneous (33%) followed by endocrine (27%) IRAEs. None of the factors studied showed association with development of IRAEs in this cohort.

Conclusion: This study reiterates that autoimmunity remains the Achilles heel of cancer immunotherapy. However, most IRAEs being mild, ICIs can be continued safely. Our results emphasize the need for further large prospective trials in risk factors for IRAE.


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Clinical and demographic profiles of a cohort of adult-onset still's disease from Eastern India

Debaditya Roy, Geetabali Sircar, Parasar Ghosh; Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Background: Adult-onset Still's disease (AOSD) is a rare multisystemic autoinflammatory disease of unknown etiology. Its varied clinical presentation and lack of diagnostic biomarker can lead to life threatening complications, such as macrophage activation syndrome (MAS), demanding an early diagnosis.

Objective: To evaluate the clinical profile on presentation, laboratory parameters, clinical course, and treatment response of patients of AOSD.

Methods: Records of 37 patients who fulfilled Yamaguchi's criteria for AOSD were analysed by retrospective chart review, at our centre between the period from 2013-2022.

Results: 37 AOSD patients diagnosed between 2013 and 2022 (25 Female; mean age 29.4 years) were enrolled in the current study. All had fever, joint pain, neutrophilic leucocytosis, high ferritin, and CRP at presentation. 25 patients (67%) had skin rash, of which Erythematous maculopapular rash was the most common type. ANA and Rheumatoid factor were negative in all. Sore throat was reported by 17 (45.9%), neck pain by 4 patients. 36 patients had hepatosplenomegaly; 17 (45.9 %) patients lymphadenopathy, 9 (24.3%) transaminitis and 5 (13.5%) serositis. 10 patients had a clinical suspicion of MAS, and a ferritin level of 10,000 or more conferred an odds ratio of 2.47 for its development. Bone marrow study was done in 21 patients (56.8%) and 3 had hemophagocytosis. Most patients treated with high dose (1 mg/Kg) with Methotrexate. 10 patients received pulse Methyl prednisolone for 3 to 5 days and 8 with Tocilizumab. 1 patient died due to complication of MAS and 2 others during follow up due to disease flare. Arthritis is the commonest manifestation in long term (16 patients) with 6 of them having joint deformities.

Conclusion: Our study describes the clinical and serological features of AOSD, and their outcomes. Our results demonstrate that higher systemic score, CRP, and ferritin levels predict worse outcomes.


  POS229 Top


Basic science

Lubna Khurshid; New City Hospital, Srinagar, Jammu and Kashmir, India

Background: Rheumatic diseases lead to public health burden in a good number of patients. Despite being a common cause of morbidity and disability leading to work loss and poor quality of life number of patients seeking specialist consultation for rheumatic disorders remains poor.

Objectives: To identify various factors leading to diagnostic and therapeutic delay so as to improve the disease outcome and quality of life.

Methods: 378 patients between age group of 5 to 82 years of either sex attending outpatient department of Rheumatology at a multispecialty hospital were studied.

Results: Mean age of patients seeking Rheumatology opinion was 40 years with 74% being females and 26% males. The most common presenting complaint was joint pains (75%) followed by backpain (34%) and musculoskeletal pains other than joint pains (25%). Miscellaneous symptoms including fatigue, decreased appetite, weight loss etc accounted for 55% cases. After comprehensive clinical workup supplemented by investigations patients were categorized into various diagnostic categories. Rheumatoid arthritis (44%) was the most common diagnosis made followed by osteoarthritis (31%). 25% patients were categorized as having non rheumatologic disorders. 74% patients had visited non-rheumatologists before presentation and only 11% sought Rheumatology opinion as the first one. Various deformities were found in 11% patients and drug related toxicities mainly glucocorticoid related were found in 17%.

Conclusion: Improving awareness and education about Rheumatologic diseases and among patients as well as clinicians can help in avoiding diagnostic and therapeutic delay and better outcome of patients with rheumatic diseases.


  POS230 Top


Diabetic myonecrosis: A single centre experience

Pooja S Rajesh, B N Shivaprasad, Srinivas Nalloor, H V Vijay, C Praveen; Apollo BGS Hospital, Mysuru, Karnataka, India

Background: Diabetic myonecrosis is a relatively rare complication of long standing, uncontrolled diabetes mellitus. Other terms used to describe the disease include diabetic muscle infarction or tumoriform focal muscular degeneration, aseptic myonecrosis and ischemic myonecrosis. Clinical features include sudden onset of non-traumatic pain and swelling, commonly affecting muscles of the anterior compartment of thigh. It is often seen in association with retinopathy, nephropathy, and neuropathy.

Methods: A retrospective study of clinical, biochemical, and radiological characteristics of patients admitted with diabetic myonecrosis at Apollo BGS Hospital over the last 5 years was done.

Results: The study includes a total of seven patients with diabetic myonecrosis, 3 female and 4 male patients with mean age at presentation of 54 years. The mean duration of diabetes was 11.8 years with mean HbA1c of 7.4%. All patients had one/more micro/macro vascular complications. Elevated serum creatinine (Mean value- 5.2 mg/dl) was noted in all patients. MR imaging revealed characteristic findings of heterogeneous hyperintensity of affected muscles in all patients. Patients showed symptomatic improvement with bed rest, analgesics, vasodilators, and good glycemic control.

Conclusions: Diabetic Myonecrosis is a rare long-term complication of diabetes that needs to be diagnosed and treated early to prevent morbidity.


  POS231 Top


Study of anxiety and depression in females of reproductive age group diagnosed with rheumatic diseases

Radhika Jakhotia, G C Yathish, Benzeeta Pinto, Abhishek Patil, K S Sreejitha, Naman Jain; Manipal Hospital, Bengaluru, Karnataka, India

Background: Reproductive aged females with rheumatic Disease are prone for anxiety and depression because of the disease, fear of immunosuppressant agents and effect of the disease and drugs on pregnancy. The purpose of this study is to describe the frequency of anxiety and depression symptoms employing the Hospital Anxiety and Depression Scale (HADS) in women in reproductive age.

Objectives: 1) To study the prevalence of anxiety and depression in females of reproductive age group diagnosed with rheumatic diseases 2) To study the factors affecting anxiety and depression in these group of patients.

Methods: Cross sectional study; Duration -July 2022- August 2022; Females of age - 18-45yrs who were diagnosed with rheumatic diseases.

Results: A total of 150 women were included. Most of them were in the age group 30-40 years (n=76 (49.7%). The most frequent diagnosis was rheumatoid arthritis 53 (34.6%), followed by systemic lupus erythematosus 28 (18.3 %). Total abnormal HADS score (higher than 7) was observed in 61 patients (40.66%). Total possible anxiety cases were 77 out of total 150 patients (51.3%) while total possible depression cases were 28 (18.6%). In rheumatoid arthritis, the most frequent abnormal HADS score (>7) was seen in 20 (37.7%) patients. Subjects who were not on DMARD biologics had total HADS higher than 7 score (n= 54 (36.0%) compared to not on DMARD biologics 7 (4.6%) and which was statistically significant (p <0.05). Total abnormal HADS score varies significantly (p < 0.05) among rheumatic diseases groups.

Conclusion: Anxiety and depression was more in reproductive age group females (Prevalence =40.6%), probably due to pregnancy planning in this group. Timely detection of these psychiatric illness will help in better management of rheumatic diseases.


  POS232 Top


Clinical profile and treatment in patients with orbital inflammatory disease: A cohort of 16 patients from a single centre in South India

Anuroopa Vijayan, Padmanabha Shenoy, Marian Pauly1, T Leka, K Narayanan, S Anu, U Raswith2; Dr Shenoys CARE, 1Giridhar Eye Institute, 2Sree Sudheendra Medical Mission, Kochi, Kerala, India

Background: Orbital inflammatory disease (OID) accounts for up to 6% of orbital diseases. The spectrum of orbital inflammatory disease ranges from specific disease diagnosis like GPA or sarcoidosis, to non-specific inflammation which may involve one or multiple structures of the orbit. Wide heterogeneity in the clinical presentation poses a huge diagnostic challenge.

Objectives: To describe the clinical features, immunological profile, and treatments in 16 patients diagnosed with orbital inflammatory disease in the last 4 years at our centre.

Methods: The details of the 16 patients including history, physical examination, laboratory, radiologic studies, and tissue diagnosis in indicated cases were analysed.

Results: A total of 16 patients were included [Table 1]. Out of the 16 patients, 11 patients (68.75%) had unilateral disease. 5 patients had extra orbital manifestation, among them 3 were diagnosed with GPA with involvement of kidney, lungs, and middle ear, 1 patient was diagnosed with possible systemic IgG4 RD with raised S IgG4 levels and had autoimmune pancreatitis, mass involving posterior mediastinum and the other 1patient was diagnosed with Sarcoidosis when she developed complete heart block. Imaging was available in 15 patients. Biopsy details were available for 10 patients. 2 patients had Isolated orbital myositis and 2 patients was diagnosed with Idiopathic OID with involvement of multiple structures in orbit.2 patients were diagnosed as Probable IgG4 related ophthalmic disease. 2 patients presented with dacryoadenitis and arthritis was diagnosed as Sjogrens. 1 patient each was diagnosed with Tolosa Hunt syndrome, Ocular lichen planus, Sclerosing dacryoadenitis. All patients had received treatment with pulse steroids or oral steroids (1 mg / kg) and steroid sparing agents with good response.
Table 1

Click here to view


Conclusion: OID encompasses a group of heterogeneous inflammatory diseases of the orbit. The challenges in OID include diagnosis through careful history, physical examination, laboratory, and radiologic investigations.


  POS233 Top


Infections complicating course of interstitial pneumonia with autoimmune features

Nishant Gautam Kamble, P M Ankush, Sayan Mukherjee, V Abilash Krishnan, Mukesh Maurya, Pankti Mehta, Puneet Kumar, Urmila Dhakad; Department of Clinical Immunology and Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Interstitial pneumonia with autoimmune features (IPAF) course is complicated by number of comorbidities, infection being one of them. An acute respiratory infection can cause rapid deterioration of an underlying Interstitial lung disease (ILD). Patients with fibrotic changes are susceptible to chronic infections like tuberculosis, Aspergillus species infections. Some patients need prophylactic antibiotics, specific therapy, and some need adjustment in immunosuppression.

Objectives: 1. To determine characteristics of patients with IPAF. 2. To determine infection profile of patients with IPAF.

Methods: 43 patients satisfying ERS/ATS 2015 criteria for IPAF were included in the study and their characteristics were recorded and analysed. Patients with definite Connective tissue disease associated ILD were excluded.

Results: Out of 43 patients, majority 36 (84%) were females, and mean age was 49 years. Diabetes and osteoporosis were present in 4 patients each and hypertension and hypothyroidism in 3 patients each. Breathlessness was present in all patients with majority (41%) had grade 2 dyspnea on mMRC scale. Among autoimmune features, Raynaud's phenomenon and inflammatory arthritis were most common (27% each). NSIP pattern on HRCT was most common pattern (81%), UIP in 16% and LIP in 2 %. Forty one out of 43 patients (95%) were positive for ANA by immunofluorescence at 1:100 dilution. Most common autoantibodies on ENA and MSA immunoblot were Ro52. During follow up period, 14 (32%) patients developed infections, which included lower respiratory tract infection (9 patients), urinary tract infection (3 patients), infective diarrhea (1 patient) and herpes zoster (1 patient). Out of 9 patients with LRTI, pseudomonas aeruginosa and klebsiella pneumoniae isolated from 3 patients each. Two had COVID 19 pneumonia, and Aspergillus fumigatus was isolated from 1 patient. In patients with UTI, E. coli was isolated from culture. One patient developed reactivation of hepatitis B on immunosuppression.

Conclusion: Patients with IPAF develop serious infections during course of illness which need prompt diagnosis and treatment. These patients are more susceptible to infections probably due to abnormal lung parenchyma, and immunosuppressive medications.




  POS234 Top


Awareness, knowledge and attitude about complementary and alternative medications among rheumatologists

Yogesh Preet Singh, Anubhav Joshi, B N Shivaprasad1, G C Yathish2, Naman Jain2; Himalayan Institute of Medical Sciences, Dehradun, Uttarakhand, 1Apollo Hospitals, Mysore, 2Manipal Hospitals, Bengaluru, Karnataka, India

Background: Complementary and Alternative Medicine (CAM) therapies include a wide spectrum of practices and products. Their popularity has been increasing and are widely used by patients. The efficacy of these interventions is unknown and some of these interventions can potentially interact with medications. Physicians are often unaware of this usage by patients of CAM.

Objectives: To evaluate rheumatologists' knowledge, attitudes, and practice patterns regarding CAM.

Methods: Rheumatologists in India were invited to complete a web-based survey.

Results: Totally 81 responses were received. The mean age was 41 years (24 - 71). The mean work experience was 12 years (1 - 44). Most (80%) were aware of different modalities of CAM. Nearly half (46.2%) of them discuss CAM with patients. Majority (80%) felt CAM usage was common by patients. Nearly 70% do not suggest CAM to the patients and 60% were not comfortable with self-usage of CAM by patients along with standard of care. The most common CAM modalities suggested by participants were oral supplements (20%), oils / ointments (28%), mind and body practices (34%). The common conditions for whom CAM was suggested were soft tissue rheumatism and degenerative arthritis. Nearly 60% felt CAM may have a role as a part of integrative or complementary medicine. Majority (87.5%) felt the need for quality literature regarding use of CAMs in rheumatological diseases and lack of such literature currently (70%).

Conclusion: Majority are aware about CAM and different modalities; and felt CAM self-usage by patients was prevalent. Most of them felt CAM may have a role as complementary or integrative medicine. But felt there was a lack of quality scientific literature regarding CAM usage. There is further need for research regarding usage of CAM and to work towards an integrated healthcare model.


  POS235 Top


Off-label use of tofacitinib in diseases other than rheumatoid arthritis and ankylosing spondylitis

Sapan C Pandya, Pooja Srivastava1, Dhaval Tanna2; Vedanta Institute of Medical Sciences, 1STAR Hospital, 2Saurashtra Rheumatology Center, Ahmedabad, Gujarat, India

Background: Tofacitinib has been FDA approved for use in RA and AS. There is emerging data about its off-label use in other rheumatologic diseases. We decided to present our data on its similar use.

Method: To analyse outcome of use of tofacitinib in other diseases like CTDs and vasculitides, and its safety and efficacy.

Results: In our series of patients where we used tofacitinib off label, best response in terms of patient and physician global was seen in Sjogren's syndrome and granulomatous diseases like granulomatous mastitis, Sarcoidosis and Takayasu's arteritis. SLE patients and MCTD did not respond very well. Musculoskeletal pains were the main reason for starting tofacitinib in these.


  POS236 Top


Adult-onset still's disease: A study of 25 cases

Sachdev Girija Rajkumar, Sunil Singh, Rohini Samant; Department of Rheumatology, PD Hinduja National Hospital and MRC, Mumbai, Maharasthra, India

Background: Still's disease is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, transient maculopapular rash, lymphadenopathy, hepatosplenomegaly, and serositis.

Methods: A retrospective analysis of adult patients with Still's disease diagnosed from 2000 to 2022 was carried out. Their clinical features and laboratory findings at presentation, disease course, and outcomes were analyzed.

Results: Data of 25 patients with Still's disease were analyzed. The age at disease onset ranged from 16 to 59 years with a mean of 32.55, of which majority were male i.e 14. The mean duration of illness from onset of symptoms to presentation was 9.8 months (range) suggestive of significant diagnostic delay. The most common clinical manifestations were fever /PUO (n=25), articular symptoms (24), rash (n=18), weight loss (n=21), and sore throat (n=8). Elevated ESR was present in all patients with a mean of 97.32 mm at 1 h. Hepatic enzymes were elevated in 11 patients at disease onset. Fever responded quite early during treatment, while arthritis tended to have a chronic, polycyclic pattern refractory to conventional DMARDs in some patients. Cyclosporine led to dramatic recovery in seven patients. One patient developed resistant arthritis which improved only with tocilizumab. Macrophage activation syndrome (MAS) occurred in two patients, one after sulfasalazine therapy, the other patient died. It was seen as a presenting manifestation in an elderly patient, who later developed cholangiocarcinoma and in another as a post Covid event.

Discussion: In this study of 25 patients with Still's disease, we have highlighted that Still's disease has a characteristic constellation of symptoms and signs and laboratory features. Fatal complications can occur rarely and should be anticipated. These observations need to be confirmed prospectively in larger number of patients from India.{Figure 89}

Conclusion: Adult Stills disease should be considered in the differential diagnosis of fever of unknown origin. Nonsteroidal anti-inflammatory drugs, steroids, and methotrexate may not be always effective, cyclosporine and tociluzimab are effective in resistant cases. Sulfasalazine should be avoided in cases of AOSD.


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IgG4-related disease – Bird's eye view of the Malefic Masquerader!

R S Anjana, Ramesh Jois, Sasikala Bheemireddy1, Vineetha Shobha1, Chethana Dharmapalaiah2, R Subramanian3, B G Dharmanad, Sathish Rao Kalanje, Uma Karjigi4; Department of Rheumatology and Immunology, Manipal Hospitals, 1Department of Clinical Immunology and Rheumatology, St. John's Medical College and Hospital, 2Department of Rheumatology, Aster CMI Hospital, 4Department of Rheumatology, Apollo Specialty Hospitals, Jayanagar, Bengaluru, 3Department of Rheumatology, JSS Medical College, Mysuru, Karnataka, India

Background: IgG4-Related disease (IgG4-RD) is a chronic indolent autoimmune inflammatory fibrosclerosing condition with potential to involve various organs. Published data on IgG4-RD from India is sparse. We present a multicentre retrospective case series from Karnataka.

Discussion: 24 patients with a diagnosis of IgG4-RD were analysed. 13 (54%) were males, 11 (45%) were females. The mean age of patients was 47yrs (26 - 82). Most common presentation was glandular involvement (n=10, 41%). 7 presented as sinonasal/orbital localized mass, 3 had renal involvement, 3 Pancreatic, 1 each had meningeal, aortic, retroperitoneal involvement. [Table 1] gives detailed account of all patients. Site-specific imaging was done in all patients. Elevated serum IgG4 levels were seen in 19 patients (79%). 19 (79%) patients underwent biopsy of the involved organ and had histopathological findings consistent with a diagnosis of IgG4-RD. Biopsy was not done in 5 (20%) patients due to inaccessibility of involved tissue or refusal to provide consent. All biopsy specimens met the recommended histopathological and immunostaining criteria for IgG4-RD. All patients received glucocorticoids and steroid sparing drugs. 8 (33%) patients received Rituximab, 7 (29%) received Methotrexate, 4 (16%) MMF, 1 each Azathioprine and Tofacitinib and 4(16%) received combination DMARDs. Organ-specific clinical improvement was seen in all patients. 1 patient succumbed to COVID.
Table 1: Clinical profile of IgG4-related disease patients

Click here to view


Conclusion: Our case series is one of the largest from India. It demonstrates the diverse clinical presentation of IgG4-RD amongst Indian patients consistent with international published literature. Most of our patients in the study were younger. Definitive diagnosis of IgG4-RD was established by histological confirmation in majority of patients. Published literature shows Rituximab as the preferred drug in IgG4-RD, while our series shows good response to multiple other steroid-sparing agents. This is encouraging and becomes important in resource-constrained countries. Retrospective nature of data and non-uniformity in treatment protocols may be the major limitations of this study.




    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16], [Figure 17], [Figure 18], [Figure 19], [Figure 20], [Figure 21], [Figure 22], [Figure 23], [Figure 24], [Figure 25], [Figure 26], [Figure 27], [Figure 28], [Figure 29], [Figure 30], [Figure 31], [Figure 32], [Figure 33], [Figure 34], [Figure 35], [Figure 36], [Figure 37], [Figure 38], [Figure 39], [Figure 40], [Figure 41], [Figure 42], [Figure 43], [Figure 44], [Figure 45], [Figure 46], [Figure 47], [Figure 48], [Figure 49], [Figure 50], [Figure 51], [Figure 52], [Figure 53], [Figure 54], [Figure 55], [Figure 56], [Figure 57], [Figure 58], [Figure 59], [Figure 60], [Figure 61], [Figure 62], [Figure 63], [Figure 64], [Figure 65], [Figure 66], [Figure 67], [Figure 68], [Figure 69], [Figure 70], [Figure 71], [Figure 72], [Figure 73], [Figure 74], [Figure 75], [Figure 76], [Figure 77], [Figure 78], [Figure 79], [Figure 80], [Figure 81], [Figure 82], [Figure 83], [Figure 84], [Figure 85], [Figure 86], [Figure 87], [Figure 88], [Figure 89]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8], [Table 9], [Table 10], [Table 11], [Table 12], [Table 13], [Table 14], [Table 15], [Table 16], [Table 17], [Table 18], [Table 19], [Table 20], [Table 21], [Table 22], [Table 23], [Table 24], [Table 25], [Table 26], [Table 27], [Table 28], [Table 29], [Table 30], [Table 31], [Table 32], [Table 33], [Table 34], [Table 35], [Table 36], [Table 37], [Table 38], [Table 39], [Table 40], [Table 41], [Table 42], [Table 43], [Table 44], [Table 45], [Table 46], [Table 47], [Table 48], [Table 49], [Table 50], [Table 51], [Table 52], [Table 53], [Table 54], [Table 55], [Table 56], [Table 57], [Table 58], [Table 59], [Table 60], [Table 61], [Table 62], [Table 63], [Table 64], [Table 65], [Table 66], [Table 67], [Table 68], [Table 69], [Table 70], [Table 71], [Table 72], [Table 73], [Table 74], [Table 75], [Table 76], [Table 77], [Table 78], [Table 79], [Table 80], [Table 81], [Table 82], [Table 83], [Table 84], [Table 85], [Table 86], [Table 87], [Table 88], [Table 89], [Table 90], [Table 91], [Table 92], [Table 93], [Table 94], [Table 95], [Table 96], [Table 97], [Table 98], [Table 99], [Table 100], [Table 101], [Table 102], [Table 103], [Table 104], [Table 105], [Table 106], [Table 107], [Table 108], [Table 109], [Table 110], [Table 111], [Table 112], [Table 113], [Table 114], [Table 115], [Table 116], [Table 117], [Table 118], [Table 119], [Table 120], [Table 121], [Table 122], [Table 123], [Table 124], [Table 125], [Table 126], [Table 127], [Table 128], [Table 129], [Table 130], [Table 131], [Table 132], [Table 133], [Table 134], [Table 135], [Table 136], [Table 137], [Table 138], [Table 139], [Table 140], [Table 141], [Table 142], [Table 143], [Table 144], [Table 145]



 

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