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   Table of Contents - Current issue
Coverpage
September 2022
Volume 17 | Issue 3
Page Nos. 225-332

Online since Wednesday, September 14, 2022

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EDITORIAL  

Comorbidities in rheumatic disorders: Time to tame the invisible gorilla p. 225
Subramanian Shankar
DOI:10.4103/injr.injr_1000_22  
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ORIGINAL ARTICLES Top

Burden of associated comorbidities in autoimmune rheumatic diseases in Indian population: An interim report based on the Indian rheumatology association database p. 227
S Chandrashekara, Padmanabha Shenoy, Uma Kumar, Sapan Pandya, Alakendu Ghosh
DOI:10.4103/injr.injr_125_21  
Aim: The present study is intended to analyze the preliminary interim data to understand the burden of associated comorbidities in autoimmune rheumatic diseases (AIRDS) in the Indian population from the Indian Rheumatology Association database. Materials and Methods: The independent prospective, multicenter, observational study evaluated the preliminary data obtained from 5 centers across India. The data pertaining to comorbidities were collected, as per the definition of the new International Classification Diseases-10 version of the Charlson Comorbidity Index. The details such as socioeconomic status, impairment in the working capability, and their capacity to cope with their job after developing the disease were gathered for selected patients. The patients were broadly classified into five based on diagnosed AIRDS namely rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), seronegative spondyloarthropathy (SpA), psoriasis arthritis (PsA), and scleroderma. The prevalence of different comorbidities was compared between the groups using Chi-square or Fischer-exact test for nonparametric data and analysis of variance for continuous variables. Results: The study considered the data of 1885 for the analysis and 129 patients were excluded due to missing variables and data inconsistency. The socioeconomic distribution of AIRDS indicated that SLE was more prevalent among upper-middle-class followed by SpA (60.3% and 50.94%, respectively). RA and systemic sclerosis (SSc) were more common among the lower-income group, and PsA was common among the upper socioeconomic group. The most common comorbidity found in patients with RA and PsA was hypertension (20.97%, 17.14%), SLE and SSc was thyroid disease (21.49%, 17.78%), and SpA was diabetes (2.96%). Conclusion: AIRDs are associated with the significant burden of comorbidities. Further studies are needed to understand the pattern of prevalence of comorbidities across different age groups.
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Translation and validation of Gujarati version of Indian HAQ in RA patients p. 234
Jaspreet Kaur Kang, Neeta J Vyas
DOI:10.4103/injr.injr_224_21  
Background: Routine assessments of disease activity and functional status play a key part in ensuring a high-quality rheumatologic care. The most widely used, Health Assessment Questionnaire (HAQ), has become a standard outcome measure of physical function in clinical studies. Aims and Objectives: The study aimed to translate the English version of Indian Version of Health Assessment Questionnaire into Gujarati language and validate the translated Gujarati version of the scale. Materials and Methods: The English version of HAQ was translated into Gujarati language using the forward-translations and back-translation method. The translated questionnaire was administered to a sample of 65 outpatients on routine follow up visit and one week later with rheumatoid arthritis (mean age) to test for content validity and reliability. Results: Internal consistency of each item was evaluated by Cronbach's alpha and the Construct validity was evaluated by determining Spearman's correlation between the Gujarati Indian HAQ score and disease activity variables. Reliability testing showed an intraclass coefficient for HAQ of 0. 848. The internal consistency was acceptable with a Cronbach's alpha of 0.91 and indicated adequate construct validity. Conclusion: Hence it is suggestive that the Gujarati version of Indian HAQ is a reliable and valid tool for evaluation of the functional status in rheumatoid patients.
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Prevalence of primary knee osteoarthritis in the urban and rural population in India p. 239
Rohit Yadav, Ajay Kumar Verma, Arjun Uppal, Hemant Singh Chahar, Jaydeep Patel, Chandra Prakash Pal
DOI:10.4103/injr.injr_337_20  
Introduction: Osteoarthritis (OA) is one of the most common causes of pain and disability, representing a significant burden for the individual and for society. The knee is one of the most prevalent among all joint sites affected by OA. The aim of this study is to analyze the prevalence of primary knee OA in the urban and rural population in India. Methods: The study was a community-based cross-sectional study. The target population was from 5 metropolis (big cities), 5 small cities near the metropolis, 5 towns, and 25 villages from blocks of these towns, i.e., 5 sample groups were covered. We got a minimum sample size (n) = 4680 which was further divided into five sites equally (936 from each site). This was rounded to a sample of 1000 from each site. Data were collected by structured questionnaires, interviews, and observation of clinical and radiological findings. OA was graded using the Kellegren and Lawrence Scale. Results: The overall prevalence of primary knee OA in big cities was 33.2%, 19.3% in small cities, 18.3% in towns, and 29.2% in villages. In the current study, it was seen that 32.7% of the population in the big city had a sedentary lifestyle as compared to 28.7% in villages and 18.1% in towns. About 44.5% of the population in the village had physically demanding work as compared to cities and towns. Conclusion: The prevalence of symptomatic primary OA knee in urban areas is much higher than reported from rural regions. To guide the distribution of health-care resources and preventive strategies in future, our findings will be useful.
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Cutaneous manifestations of rheumatoid arthritis: An observational study from a Tertiary Care Hospital in Eastern India p. 244
Partha Sarathi Nayak, Kakali Mridha, Pradyot Sinhamahapatra, Biswanath Naskar, Gobinda Chatterjee, Adrija Datta
DOI:10.4103/injr.injr_156_21  
Background: Rheumatoid arthritis (RA) has both articular and extra-articular manifestations, with certain dermatoses occurring in increased frequencies in this population. Since clinicodemographic profile varies according to ethnicity and geographic location, the present study was conducted to record the dermatological presentations of RA in Eastern Indian population. Materials and Methods: This was an observational cross-sectional study conducted in a tertiary care hospital in Eastern India on adult patients of either sex attending the department of Dermatology. Consecutive patients were recruited and detailed clinicodemographic profile was recorded in a pretested, semi-structured pro forma. Results: Three hundred and twenty-four consecutive patients with a female predominance (M: F = 1:3.3) who fulfilled the eligibility criteria were recruited. Thirty-six patients had more than one cutaneous manifestation. Three hundred and sixty-two dermatological manifestations were noted with 57 (15.7%) having specific findings, 14 therapy-related adverse effects and 291 (80.4%) nonspecific findings. Vascular changes were commonest (22; 6.1%) followed by rheumatoid nodules (3.3%) and leg ulceration (2.8%). Rheumatoid factor was found positive in 233 (71.9%) cases and anti-citrullinated peptide antibody (ACPA) was positive in 250 (77.2%) cases. There was statistically no significant association between specific dermatological manifestations and age, duration, and family history of RA, rheumatoid factor, and ACPA positivity. Conclusion: Awareness and recognition of various skin manifestations is essential for a concerted therapeutic approach to patients with RA.
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Translation, cross-cultural adaptation, and validation of fatigue severity scale into Bangla in patients with systemic lupus erythematosus p. 250
Imam Gazzali, Md Abu Shahin, Ariful Islam, Iftekhar Hussain Bandhan, Sumayia Minhaj, Mohammad Moniruzzaman, Sabrina Yesmin, Minhaj Rahim Choudhury, Syed Atiqul Haq, Mohammad Mostafa Zaman
DOI:10.4103/injr.injr_225_21  
Objective: Fatigue is common in patients with systemic lupus erythematosus (SLE). Fatigue severity scale (FSS) is used to measure fatigue. This study was aimed to develop a culturally adapted Bangla version of the English FSS and to validate it. Methods: English version of FSS was translated into Bangla and adapted in the local sociocultural context, following the recommendations of Beaton et al. One hundred and twenty SLE patients were assessed in the Rheumatology Department of Bangabandhu Sheikh Mujib Medical University. Content validity of the adapted Bangla version was evaluated by item-level content validity index (I-CVI) and scale-level content validity index (S-CVI). Correlation between FSS and health assessment questionnaire-disability index (HAQ-DI) and 12 item short form health survey (SF-12) was measured for the assessment of convergent validity. Internal consistency and test–retest reliability were also assessed. Results: Our procedure had no problem with the content and language. I-CVI and S-CVI for each item was one. FSS showed strong positive correlation (>0.7) with HAQ-DI and strong negative correlation (>−0.7) with SF-12. The scale demonstrated excellent internal consistency (Cronbach's alpha = 0.93) and test–retest reliability (intraclass correlation coefficient = 0.93). Conclusions: The adapted Bangla version of FSS is a reliable and valid instrument and can be recommended for the self-assessment of fatigue in SLE patients.
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Clinical profile and outcome of antineutrophil cytoplasmic antibody-associated vasculitis: A retrospective observational study from South India p. 255
Sham Santhanam, Hema Murugesan, Kavitha Mohanasundaram
DOI:10.4103/injr.injr_245_21  
Aim: The aim was to study the clinical profile and outcome of patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This was a retrospective observational study of patients with a diagnosis of AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], and eosinophilic granulomatosis with polyangiitis [EGPA]). Patient records from January 2015 to December 2020 were retrieved and included in the analysis. We used the European Medicines Agency (EMA) algorithm for classification of patients. Results: Forty (14 males and 26 females) patients were included (34 – GPA, 4 – MPA, and 2 – EGPA). The median age was 48 years (54.5 – males and 46 – females). The median disease duration was 6 months, and the median follow-up duration was 18.5 months. As per European Vasculitis Society disease categorization, 25 had organ/life-threatening type, 10 had rapidly progressive renal failure/pulmonary hemorrhage, 4 nonorgan threatening, and 1 refractory disease. The median erythrocyte sedimentation rate (mm/h) and C-reactive protein (mg/L) were 64 and 52.49, respectively. The median Birmingham Vasculitis Activity Score was 15.5. Twenty-six had constitutional features, 22 musculoskeletal, 24 pulmonary, 19 renal, 13 ocular, 13 ENT involvement, 10 mucocutaneous, 11 peripheral nervous system, 1 central nervous system, and 3 had cardiovascular involvement. As induction therapy, cyclophosphamide was used in 15 and rituximab in 20 patients. Conclusion: The age of onset of AAV was relatively delayed in comparison to other Indian studies. We had female predominance similar to other Indian studies but in contrast to European and US cohorts. In comparison to other Indian studies, prevalence of ENT and ocular manifestations were less and none of the subjects had gastrointestinal involvement.
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BRIEF REPORT Top

Characteristics and diseases associations of tuberculosis in lupus: A retrospective single-center analysis p. 264
Dunga Sai Kumar, Sanket Ishwarbhai Shah, Chengappa Kavadichanda, Aishwarya Gopal, Prakash Babu Narasimhan, Vir Singh Negi
DOI:10.4103/injr.injr_153_21  
Objective: The objective of this study is to identify factors associated with active tuberculosis (TB) in patients with systemic lupus erythematosus (SLE) and to study the impact of TB on SLE disease activity. Methodology: We screened case records of 643 individuals with SLE for the diagnosis of TB. SLE patients with TB (cases) (n = 29) were matched for age, sex, and disease duration in the ratio of 1:3 with controls (n = 94) (SLE without TB). Results: Pulmonary TB was seen in 41% (n = 12) and extrapulmonary TB in 59% (n = 17). Musculoskeletal TB (n = 6; 34.2%) was the most common extrapulmonary manifestation. TB was seen among those with lupus nephritis (55% vs. 38.4%) (P < 0.005) and those with autoimmune hemolytic anemia (48% vs. 38%). A higher median dose of daily glucocorticoids 11.5 (7.5–30) in 1 month preceding TB diagnosis was associated with a higher incidence of TB. Upon multivariable analysis, 1 month mean daily dose of glucocorticoids ≥ 7.5 mg (odds ratio [OR], 1.47; 95% confidence interval [CI], 1.07–2) was independently associated with TB. On a follow-up duration of 16 months, SLE flare was more among those with TB as compared to the controls (OR-2.49 95%; CI-1.39–4.48; P = 0.002). Conclusion: Extrapulmonary TB is the most common form in individuals with SLE. A higher steroid dose seems to predispose to TB infection. TB infection may increase the risk of SLE disease flare.
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REVIEW ARTICLES Top

The association of foot posture and osteoarthritis of the medial compartment of the knee joint: A literature review p. 270
Shrikant Wagh, Rizwan Razak
DOI:10.4103/injr.injr_39_22  
Osteoarthritis (OA) of the knee joint (KOA) is a major cause of morbidity among older adults. Medial compartment KOA (MCKOA) is the more common form. High body mass index (BMI), obesity, and older age are some of the risk factors for KOA. Adult acquired pronated foot deformity is another common condition with some common risk factors. This systematized review aims to assess the association of MCKOA and posture of the foot and the impact of assessment of foot posture in the management of MCKOA. A search was carried out from PubMed, Cochrane, and Google Scholar for studies related to the association of foot posture and MCKOA. 11 studies (out of 5926) that met the inclusion criteria were selected for analysis. These studies (3685 participants) were divided into two subgroups: (a) studies of association of foot posture with MCKOA (n = 8) and (b) studies of association between foot pain and knee pain/osteoarthritis (n = 3). The studies are heterogeneous due to different methods of foot posture assessment, KOA diagnosis, and noninclusion of covariates such as age, gender, and BMI. These studies indicate that there is an association between pronated foot posture and MCKOA. There is insufficient evidence to determine the impact of foot posture assessment in the management of MCKOA. An assessment of foot posture followed by correction with customized orthoses may help relieve pain in some of these patients. It is, therefore, essential to assess the foot posture in all patients of KOA.
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Anti-inflammatory effects of photobiomodulation in the management of osteoarthritis of the temporomandibular joint: A systematic review of animal studies p. 279
Virginia Bautista, Sandeep Talluri, Olga A Korczeniewska, Dimitrios Michelogiannakis, Junad Khan
DOI:10.4103/injr.injr_192_21  
Aim: There is a controversy in regards to the efficacy of photobiomodulation (PB) in the management of osteoarthritis (OA) of the temporomandibular joint (TMJ). The aim of this study was to assess if PB induces anti-inflammatory effects for the management of OA of the TMJ. Materials and Methods: The focused question was “Does PB induce anti-inflammatory effects for the management of OA of the TMJ?” Indexed database search was performed up to and including June 2020. Data regarding study design, study grouping, subjects, age, sex, OA induction method, outcome variables, and parameters regarding OA in TMJ were extracted. Results: A total of 47 articles were initially identified through indexed databases and 6 experimental animal studies were included in the present review. Overall the risk of bias was high in two studies and moderate in four studies. A variety of results were evaluated after histopathological, biochemical, and radiographic analysis. After PB, there was a reduction in the articular disc thickness in the anterior, middle, and posterior regions, inflammation in the retrodiscal tissues was significantly reduced, and there was reduced expression and activity of matrix metalloproteinase (MMP)-2 and MMP-9, glycosaminoglycans and caspase-3 on biochemical analysis of the TMJ. The condyle showed the absence of inflammation after low-level laser therapy, and there was also a reduction of inflammatory infiltrate in the TMJ. Conclusions: Within the limits of the present systematic review, it is concluded that PB is a potent therapeutic agent in the management of OA in TMJ.
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CASE BASED REVIEW Top

Intrauterine onset of multisystem inflammatory syndrome in a neonate temporally associated with maternal COVID-19 p. 289
US Nitya, Brinda M Devassy, Shiji Joseph, AP Anila, Rupesh George, VK Sreenivasan
DOI:10.4103/injr.injr_4_22  
Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is a rare but often life-threatening clinical entity that presents 3–6 weeks after SARS-CoV-2 infection with high fever, organ dysfunction, and strongly elevated markers of inflammation. Unlike MIS-C, where SARS-CoV-2 infection and multisystem inflammation occur in the same subject, maternal SARS-CoV-2 infection may cause a similar hyperinflammatory syndrome in neonates called multisystem inflammatory syndrome in neonates (MIS-N) due to transplacental transfer of antibodies. The clinical profile of these babies remains obscure due to a lack of published literature. Our case highlights the need for practicing pediatricians to be vigilant and to have a high index of clinical suspicion of MIS-N in all critically ill neonates irrespective of antenatal COVID-19 status of mother.
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Two cases of adult-onset de novo immunoglobulin A vasculitis with nephritis: Post-Covishield (ChAdOx1 nCoV-19; Oxford–Astra Zeneca) vaccination p. 294
Vijoy Kumar Jha, Ramanjit Singh Akal, Debasish Mahapatra, S Harikrishnan, Gurpreet Kaur Walia
DOI:10.4103/injr.injr_85_22  
With the ongoing worldwide COVID-19 vaccination programs, new-onset glomerular disease and relapse of the preexisting glomerular disease have been reported after COVID-19 vaccines administration. These incidences are overall very rare and had just temporal association with vaccination. It is, therefore, the causal link with the COVID 19 vaccine is not firmly established. In this case-based review, we present two cases, who presented with purpuric rashes and joint pain between 2 and 3 weeks of 2nd dose of Covishield (ChAdOx1 nCoV-19; Oxford–Astra Zeneca) vaccination. Routine evaluation in both these cases revealed significant proteinuria and microscopic hematuria. The diagnosis of immunoglobulin A (IgA) vasculitis with nephritis was established with renal biopsy suggestive of IgA nephropathy and skin biopsy findings of leukocytoclastic vasculitis. Both these cases had severe renal involvement and responded to oral glucocorticoids after 8–16 weeks of treatment. Close observation and careful monitoring of these cases are required to determine the incidence of de novo or recurrence of glomerular disease postvaccination, the need for immunosuppressive therapy, response to aggressive treatment, and long-term clinical outcomes.
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Monoclonal antibodies to treat COVID-19 in rheumatoid arthritis: A case report and a clinical appraisal of selected drug trials p. 300
Arvind Chopra
DOI:10.4103/injr.injr_5_22  
The current report describes successful treatment of a rapidly progressive severe breakthrough COVID-19 in a female physician, a known case of rheumatoid arthritis (RA). She received monoclonal antibody (Mab) combination cocktail (Casirivimab™ plus Imdevimab™) infusion on day 4 after the onset of symptoms. RA was in prolonged remission with tofacitinib. She had completed vaccination 6 weeks earlier and worked in a COVID hospital. Post infusion, there was substantial improvement, and she was discharged after 3 days. However, she required intermittent domiciliary oxygen for a fortnight. Fatigue and ageusia persisted for 5 weeks. Several Mabs were recently approved for emergency use in mild-to-moderate ambulant COVID-19 patients. Controlled drug trials confirmed excellent efficacy and safety. Selected data on clinical relevance and limitations are currently described. RA is susceptible to COVID-19, and some vaccines may be less effective. Intervention with Mabs ought to be judicious and timely.
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The weeping heart, the choked lung, the blinded eye, and the crying femur: A saga of juvenile dermatomyositis Highly accessed article p. 306
Arun Hegde, Suchi Acharya, Subramanian Shankar, Abhishek Kumar, Uday Bhanu Kovilapu
DOI:10.4103/injr.injr_51_21  
Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children that primarily involves the skeletal muscles and skin. However, it can manifest with a diverse range of clinical features across multiple organ systems. JDM vasculopathy causing optic neuritis and medullary bone infarcts in children are rare manifestations of JDM and have been seldom reported in literature. Similarly, cardiovascular manifestations in the form of refractory myocarditis leading to congestive cardiac failure during the early course of the disease have also been rare findings. Early diagnosis and aggressive management of such complications using biological disease-modifying synthetic antirheumatic drugs has significantly reduced the mortality and morbidity associated with JDM. Herein, we report a mini case series of three patients with JDM who presented with these rare systemic manifestations and achieved favorable outcomes while being managed using the Childhood Arthritis and Research Alliance-Treatment Protocols.
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CASE REPORTS Top

The eye of the wolf p. 311
Sahana Baliga, Samyak Mulkutkar, C Balakrishnan
DOI:10.4103/injr.injr_3_22  
Systemic lupus erythematosus is a chronic multisystemic disease with ocular manifestations seen in up to 90% of patients during their disease course. Ophthalmic manifestations can be due to disease activity or complications of therapy. Detailed history and careful examination are essential to differentiate between the entities. Timely accurate diagnosis and prompt treatment can save vision. We report two patients, one with lupus activity and the other with ocular infection to bring out the nuances in the management of such patients.
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Tjalma syndrome: A rare manifestation of systemic lupus erythematosus p. 313
Ashwaq Alhmoudi, Ahlam Almarzooqi, Maryam Alahmad, Amna Al Muhairi, Sehriban Diab, Raven Haan, Khalid Elsayed
DOI:10.4103/injr.injr_70_22  
Tjalma syndrome, also known as pseudo-pseudo Meigs' syndrome, is a rare manifestation of conditions in patients with systemic lupus erythematosus (SLE). The syndrome is characterized by the presence of ascites, pleural effusion, and an elevated cancer antigen-125 (CA-125) level. We present the case of a 27-year-old female patient admitted in 2021 without any comorbidities, who presented with unintentional weight loss, gastrointestinal upset, and ascites. Further evaluation showed elevated CA-125 levels and pleural effusions, with no atypical cells. The patient was initially treated with antiemetics, intravenous fluids, antibiotics, and total parental nutrition with no improvement in her symptoms or laboratory parameters. The results of an autoimmune workup met the criteria for the classification of SLE. After extensive investigation, she was diagnosed with Tjalma syndrome. She was subsequently treated with corticosteroids and hydroxychloroquine, resulting in the rapid resolution of the patient's nausea and emesis, and discharge from the hospital. Her ascites resolved over 4 weeks. Prednisolone was tapered down and azathioprine was added as a steroid-sparing agent. The patient eventually had complete remission of her symptoms, as well as remarkable improvements in her laboratory results. However, 8 months after her initial diagnosis, the patient showed signs of increasing SLE activity with lupus nephritis, anemia, and leukopenia, despite being compliant with her treatment regimen. The patient was initiated on oral prednisolone (1 mg/kg) and azathioprine was replaced with mycophenolate mofetil, which resulted in significant improvement of clinical and laboratory parameters within 3 weeks. Cases of Tjalma syndrome, and specifically, this presentation, are rarely reported in the literature. We present this condition to raise awareness about both the presenting symptoms and therapeutic options for Tjalma syndrome.
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IMAGES IN RHEUMATOLOGY Top

Half-filled or half-empty: Conundrum of “Hypopyon Sign” in pustular psoriasis p. 318
Kunal Chandwar, Prasanna Dogga, Juhi Dixit, Digvijay Ekbote, Rasmi Ranjan Sahoo, Anupam Wakhlu
DOI:10.4103/injr.injr_279_21  
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Accessory sacroiliac joints: A commonly misinterpreted mimic of spondyloarthropathy p. 320
Shivraj Padiyar, Prathyusha Manikuppam, Madhavi Kandagaddala, Rosh V Georgy, John Mathew
DOI:10.4103/injr.injr_44_22  
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LETTERS TO EDITOR Top

Post-COVID arthralgia p. 322
Ganesh Singh Dharmshaktu
DOI:10.4103/injr.injr_68_22  
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New-Onset rheumatoid arthritis following ChAdOx1 nCoV-19 vaccine administration p. 323
Bhupen Barman, Debashis Priyadarshan Sahoo, Alvin Khangembam, Muhammad Jamil, Pranav Ish
DOI:10.4103/injr.injr_51_22  
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The need for recreating rheumatic disease biology in a dish p. 325
Sakir Ahmed, Soumya R Mohapatra
DOI:10.4103/injr.injr_249_21  
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Systemic lupus erythematosus with extensive brain calcification p. 327
Sham Santhanam
DOI:10.4103/injr.injr_95_21  
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Simple alteration of light microscope to detect synovial fluid crystals p. 329
Manesh Manoj, Rasmi Ranjan Sahoo, Anupam Wakhlu
DOI:10.4103/injr.injr_28_22  
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BOOK REVIEW Top

The wellness guide to arthritis p. 331
Vinod Ravindran
DOI:10.4103/injr.injr_109_22  
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