Background: The elastography measurements in rheumatoid arthritis (RA) without carpal tunnel syndrome (CTS) are the least studied. Hence, the present study assessed the role of elastography in detecting the median nerve changes before the development of CTS in subjects with RA. Methodology: A prospective observational study was conducted from January 2020 to July 2021 among 112 patients with and without RA. They were divided into Group A cases and Group B controls of 56 each. The patients underwent ultrasonography and strain elastography. Changes in median nerve stiffness were the primary outcome variable. P < 0.05 was considered significant and was analyzed using CoGuide software. Results: The mean age of the patients was 48.5 ± 13.31 years, and most of them were females (64.29%) in both groups. The cross-sectional area (CSA) of median nerve at carpal tunnel inlet in cases and controls was 0.110 (0.105–0.116 cm²) and 0.090 (0.083–0.095 cm²) in right hands, respectively, and 0.109 (0.104–0.116 cm²) and 0.089 (0.082–0.093 cm²) in left hands, respectively. The mean strain ratio (SR) of the median nerve in cases and controls was 2.84 (2.41–2.98) and 1.65 (1.41–1.94) in right hands, respectively, and 2.76 (2.45–3.04) and 1.66 (1.43–1.93) in left hands, respectively. The difference in means of CSA and SR was significant across the groups (P < 0.001). Conclusion: The use of elastography in evaluating the median nerve changes in RA subjects without CTS was useful as changes in the CSA and SR were significant in cases compared to controls.

Background: Clinical patterns and disease activity burden of psoriatic arthritis (PsA) varies in different parts of the world. There are limited studies from the Indian subcontinent. Aims: To study the cutaneous and articular profile of PsA and describe their disease activity with validated outcome measures. Methods: Karnataka psoriatic arthritis cohort study is a multicenter, prospective, cross-sectional, observational study which included all consecutive PsA patients defined by Classification criteria for psoriatic arthritis (CASPAR) from 18 rheumatology centers. Results: A total of 549 PsA patients (M: F: 6:5), mean age 38.4 (±14) years were included. PsA preceded psoriasis in 81 (14.7%) while simultaneous onset was noted in 117 (21.5%). Plaque lesions (330 [78.8%]) and scalp (210 [49.2%]) were the most common type and site. Mild, moderate, and severe skin disease was noted in 480 (80%), 50 (9.3%), and 57 (10.6%) patients, respectively. Mean disease activity in PsA (DAPSA) was 18.8 (16.6); 100 (19.9%) were in remission. Low, moderate, and high joint disease activity was found in 145 (28.8%), 137 (27.2%), and 123 (24.5%), respectively. There was no correlation between skin and joint disease. Polyarthritis (216 [40.7%]) and oligoarthritis (202 [38.1%]) were the most frequent PsA subtypes. Those with a higher DAPSA (>28) were older (P = 0.02), had a shorter duration of psoriasis (P = 0.02) and higher psoriatic area and severity index scores (P = 0.0001). Conclusions: We report high articular disease activity in half while cutaneous disease activity was minimal in majority.

Background: Programmed cell death 1(PD-1)/programmed cell death ligand 1 (PD-L1) pathway is an immune checkpoint implicated in immune tolerance and involved in the pathogenesis of several autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple immune dysregulation. This study aimed to determine PD-1 and PD-L1 expressed levels on both CD3 T and CD19 B lymphocytes in SLE patients compared to healthy donors and their associations with the clinical data and disease activity of those patients. Patients and Methods: A total of 25 healthy donors and 80 SLE patients were involved in the study. PD-1 and PD-L1 expressed levels on each of CD3 T and CD19 B lymphocytes were determined in the peripheral blood (PB) using flow cytometry. Results: The expressed levels of PD-1 and PD-L1 on both CD3 T and CD19 B lymphocytes were significantly higher in PB of SLE group than that of controls (P = 0.01, P = 0.001, P = 0.009, and P = 0.001). Significant positive associations were found between PD-1 and PD-L1 expressions on both CD3 T and CD19 B lymphocytes with disease activity in SLE group (P < 0.05). Conclusion: PD-1 and its ligand PD-L1 could have a role as regulators for immune activation in patients with SLE.

Background: Early detection of retinal toxicity in patients on chronic usage of hydroxychloroquine (HCQ) and thereby the prevention of irreversible visual loss is an important goal. Methods: We performed a comprehensive ophthalmologic examination, visual field testing and macular scanning using spectral-domain optical coherence tomography (SD-OCT) and Humphrey Visual Field (HVF) 10-2 perimetry in chronic HCQ users (>1 year). Participants were grouped based on the duration of HCQ exposure (<2 years, 2–5 years; and >5 years) and compared all the ophthalmologic parameters between the groups. Results: We included 110 patients (68 rheumatoid arthritis and 42 systemic lupus erythematosus), mean age being 38.5 ± 8.9 years, and median cumulative HCQ dose was 292 (interquartile range: 146,438) g. There were significant differences in parafoveal and perifoveal thickness between the three study groups <2 years (n = 42), 2–5 years (n = 55), and >5 years (n = 13) (P < 0.05) which were evident as early as 2 years of usage. Further, the OCT parameters showed a significant correlation with perimetry changes (P < 0.001), macular thinning, color vision, and fundus changes. Conclusion: We demonstrate that SD-OCT and HVF 10-2 perimetry are complementary to each other and can be used to detect early retinal toxicity as early as 2 years of HCQ exposure.

Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease, which is associated with an increased risk of cardiovascular disease (CVD). Metabolic syndrome (MetS) is a set of factors that increase the risk of CVD, and recent studies suggested an increased prevalence of MetS in RA more than in general population. Aim: The aim of this study is to determine the prevalence of MetS in patients with RA and to evaluate its association with the disease activity and inflammation parameters. Then, to evaluate other cardiovascular risk factor and HeartSCORE Eular 2015 and its relation with the MetS. Methods: It is a cross-sectional study of 103 RA patients assessing the prevalence of the MetS in RA patients. MetS was defined according to the National Cholesterol Education Program/Adult Treatment Panel III guidelines. RA disease activity was assessed with CDAI, SDAI and DAS28 scores. Independent risk factors for MetS in RA patients were identified by logistic regression. Results: The mean age was 53 ± 10 years. The sex ratio (Male/female) was 0.3. About half of patients (45%) had moderate disease activity with a mean DAS28 CRP score of 3.9±1.38. Prevalence of MetS was 38.8% in RA patients. MetS was associated with higher age (P=0.03), late onset disease age (P=0.01), family history of CVD (P=0.016), current use of corticoid (P=0.01) and menopause status (P=0.006). However disease activity, inflammation or treatment were not associated with MetS. Conclusions: MetS in RA patients was not associated with inflammation and disease activity.

Background: Shoulder pain is considered a disabling problem and is a frequent reason for consultation in general practice. The prevalence of shoulder pain has been reported to range from 7% to 36% of population. Platelet-rich plasma (PRP) releases cytokines, which are delivered of the injury to facilitate healing. Hyaluronic acid (HA) has a lubricant and anti-inflammatory effect. The aim of this study was to compare the effect of PRP versus HA in the treatment of chronic shoulder pain. Patients and Methods: This study was a prospective randomized trial on patients with chronic shoulder pain. Fifty patients were represented into two groups based on injected material: PRP group and HA group, assessment done before and after injections (4 weeks and 6 months) by using Visual Analog Scale (VAS), constant score (CS), and Shoulder Pain and Disability Index (SPADI). Results: The study results showed that at 4 weeks after injection, the PRP group had a significantly higher Constant score in comparison to the HA group (60.91 ± 3.89 vs. 57.38 ± 6.22, P = 0.02), but no difference was at baseline and after 6 months. Furthermore, both groups had insignificant differences as regards baseline SPADI (58.77 ± 2.89 vs. 60.64 ± 3.51; P = 0.06) and 6-month follow-up after injection SPADI (55.44 ± 4.71 vs. 57.12 ± 4.40; P = 0.19), but at 4 weeks after injection, the PRP group had significantly lower SPADI in comparison to the HA group (53.52 ± 3.22 vs. 55.36 ± 2.98; P = 0.04). Conclusion: Both PRP and HA groups showed statistically significant better outcomes in VAS, CS, and SPADI during follow-up visits, but PRP was found to be superior to HA at 4-week follow-up post injection.

Background: Diffuse alveolar hemorrhage (DAH) in systemic lupus erythematosus (SLE) is not very common but is associated with high mortality. No studies from India report on DAH in SLE. Materials and Methods: From the electronic database of SLE patients, data of those with unequivocal DAH between January 2008 and March 2017 were retrieved. Clinical, laboratory, treatment, and outcome details were noted. Univariate analysis and multivariate analysis were carried out with survival as an outcome measure. Results: Twenty-four (1.31%) of 1828 SLE patients had DAH. Ten patients had DAH at diagnosis of lupus. The median interquartile range age was 23 (19.7–30) years, the median duration of hospital stay was 14 (10–22) days, and the mean SLE disease activity index was 19.5 ± 8.29. The most common clinical symptom was dyspnea followed by hemoptysis. Nephritis was the most common extrapulmonary organ involvement in 18. Hypocomplementemia was noted in 19/20, double-stranded DNA positivity in 20 patients. The mean erythrocyte sedimentation rate (ESR) was 62.17 ± 39.92 mm 1^st h. Concomitant infection was seen in eight patients and raised serum procalcitonin in four/15 patients. All patients received intravenous (IV) methylprednisolone pulses, cyclophosphamide in 20, IV immunoglobulin in 4, rituximab in 2, and plasmapheresis in 2. Twelve patients (50%) died. High ESR and mechanical ventilation were independent risk factors for mortality in patients with DAH. Conclusion: DAH in SLE is infrequent but is often associated with infection and high mortality. Mechanical ventilation and high ESR are associated with higher mortality.

Background: Semaphorin-3A (Sema-3A) is an important immunoregulator protein; it has a role in the maintenance of self-tolerance, so it is thought to be involved in the pathogenesis of many autoimmune diseases such as systemic lupus erythematosus (SLE). The purpose of the study is to assess the possible role of serum Sema-3A level as a potential biomarker for disease activity in patients with SLE and its relation with lupus nephritis. Patients and Methods: We recruited fifty SLE patients and 25 healthy controls. According to the SLE disease activity index (SLEDAI), patients were divided into two groups; active SLE (n = 25) and inactive SLE (n = 25). Sema-3A level was assessed in the study groups using enzyme-linked immunosorbent assay. Laboratory work included antinuclear antibodies, anti-ds-DNA, C3, C4, C-reactive protein, and erythrocyte sedimentation rate (ESR). Results: Serum Sema-3A level was significantly lower among SLE patients compared to healthy controls (18.14 ± 5.77 vs. 65.72 ± 38.08, P < 0.001). Moreover, it was lower among active SLE group compared to inactive group (14.96 ± 4.27 vs. 21.32 ± 5.17, P < 0.001). Serum level of Sema-3A negatively correlated with SLEDAI (P ≤ 0.001) and ESR (P = 0.006) where it was correlated positively with C3 (P ≤ 0.001) and C4 (P = 0.017). Conclusion: SLE activity is associated with decreased serum level of Sema-3A, thus it is suggested that Sema-3A is a candidate to become a useful marker for SLE disease activity.

Background: India, with its aging population is going to face a major health-care burden due to osteoarthritis (OA) knee in the coming decades. Total knee replacement (TKR) is the most effective way of treating severe OA knee and has a wide variation in its utilization. Objective: The objective of this survey was to gain insight into the factors that influence the treating doctor in considering TKR for a patient and barriers to its utilization. Methods: Rheumatologists, orthopedicians, and physicians were invited to complete a 14-item web-based survey. Results: A total of 217 variable responses were received. The majority were rheumatologists (n = 143; 67.45%). Most felt that TKR was underutilized (n = 134; 62.9%). Functional demands of the patient were the most important patient factor which influenced TKR referral (84%; n = 178). Annual income and availability of health insurance were important socioeconomic factors influencing TKR referral by 105 (50.5%) and 73 (35.1%) respondents, respectively. Financial status (n = 76; 35.8%) was an important patient-related factor influencing TKR decision. High cost was considered the biggest barrier for the underutilization of TKR by 117 (55.5%). Price regulation on knee implants had mixed views, with 94 (46.3%) saying that it had improved access of TKR to a larger population and 90 (44.3%) saying it had not had any impact. Conclusion: High cost and the nonaffordable population remain the biggest barrier to utilization of TKR in OA knee.

Background: In antinuclear antibody (ANA) testing, antidense fine speckled 70 (DFS70) antibodies are commonly detected as a specific indirect immunofluorescence assay pattern (IIFA in HEp-2-based substrates) or as bands/blot lines in immunoblotting. A negative association between anti-DFS70 antibodies and the presence of autoimmune diseases is often reported. However, some studies found that this may not be true in females. We conducted this study to see the positivity rate of anti DFS70 antibodies and their association with other autoantibodies in the predominantly hilly population of our state. Methods: Serum samples sent for ANA testing between February 2018 and December 2020 were included. ANA IIFA-positive cases underwent immunoblot profiling with 16 targets (including DFS70) blotting kit. Those with DFS70 band in the immunoblot) underwent tests for other autoantibodies such as antineutrophil cytoplasmic antibodies, and anti-dsDNA. Association analysis was performed in a software package (SPSS version 23). Results: Out of 1770 samples, about 15.3% yielded a meaningful IIFA pattern. About 10.7% of ANA positives (1.6% of total samples) had the DFS70 band on immunoblotting. Isolated DFS70 was found in the predominantly male population (6 out of 7), while most females had this antibody in the presence of other autoantibodies (17 of 22). Conclusion: Male population had predominantly isolated anti DFS70 antibody detection (with the likely possibility of using it as a marker for the absence of autoimmune disease), while in females, mostly, it was along with other autoantibodies.

The subglottic space at the cricoid level is the narrowest part of the airway. Subglottic stenosis (SGS) is generally benign and may be due to a variety of diseases, but post-intubation injury is the most frequent cause. Other causes are tracheal infections including bacterial tracheitis, tuberculosis, histoplasmosis and diphtheria, and collagen vascular diseases including granulomatosis with polyangiitis, relapsing polychondritis, etc., SGS may be a presenting complaint in rheumatological disorder. In this case series, we shall discuss patients with rheumatic diseases presenting with SGS.

The current study conducted a critical review on available clinical evidence for topiroxostat, indicated for managing hyperuricemia in patients with or without gout. Relevant observational studies related to hyperuricemia and topiroxostat published between 2014 and 2022 were retrieved from various electronic databases based on the inclusion and exclusion criteria and using different combinations of MeSH terms. The systematic search yielded 2094 articles from various databases. Based on the inclusion and exclusion criteria, 16 articles were selected for systematic review. The review considered 16 studies on serum urate-lowering efficacy of topiroxostat and 6 studies that had evaluated the efficacy of the drug in hyperuricemia patients with chronic kidney disease. Four studies that evaluated hyperuricemia patients with cardiovascular risk and 10 studies that had evaluated adverse drug reactions linked to topiroxostat use were also considered. The literature findings validated the safety, efficacy, renoprotective effects, and reduced adverse events conferred by topiroxostat. The most common adverse event noted was gouty arthritis of mild to moderate in severity. The advantages of the drug when compared to the currently available xanthine oxidoreductase inhibitors include unique dual mechanism of action, cardiac safety, and renal protection.

Ultrasonography (US) emerged as a useful imaging tool in the diagnosis of gout. However, its accuracy is still unclear. We conducted a systematic review to evaluate the performance of US in diagnosing gout. We systematically reviewed PubMed database, and all the references of eligible articles were manually screened for additional relevant papers. Studies were included if they (1) examined the performance of US in diagnosing gout, (2) used a cross-sectional design in patients presenting with acute or chronic arthritis where gout was suspected, and (3) confirmed the diagnosis of gout using microscopic identification of monosodium urate crystals as the gold standard. Seven studies were included in the present systematic review. We evaluated the diagnostic properties of three typical US signs of gout: double contour (DC), tophi, and aggregates. The sensitivity and specificity of DC for gout varied, respectively, from 42% to 87.8% and 64.1% to 97%. The sensitivity of tophi for gout was between 19% and 46% and its specificity between 93% and 100%. The sensitivity and specificity of aggregates for gout ranged, respectively, between 30.3% and 78.9% and 65% and 90.9%. When any of these US features was present, the sensitivity for the diagnosis of gout increased up to 96% while the specificity decreased to 68%. Inversely, when all three signs were observed, the specificity tended to 100% but with a poor sensitivity of 17%. US had high specificity for the diagnosis of gout in patients presenting with undifferentiated arthritis. Its sensitivity depends on which US signs are taken into account and the joints being assessed.

Owing to the difficult anatomy, difficulty in procuring tissue samples, and clinical mimicry, the diagnosis of extrapulmonary, especially osteoarticular tuberculosis (TB), remains a challenge. Arriving at the correct diagnosis of articular TB frequently requires synovial biopsy and synovial fluid or tissue culture. Efficient utilization of rapid molecular assays such as Xpert Mycobacterium tuberculosis (MTB)/RIF assay can support the timely and accurate diagnosis. Xpert® MTB/RIF (Xpert) is a rapid, automated, nucleic acid amplification assay, recommended by the World Health Organization for the simultaneous detection of MTB complex and rifampicin resistance. Here, we report the case of long-standing inflammatory monoarthritis wherein the diagnosis took a protracted course. This case is reported to highlight that the employment of highly specific modern diagnostic technologies which are available through the Revised National TB Control Program results in swift and accurate diagnosis.

Both Takayasu arteritis (TAK) and ulcerative colitis (UC) are uncommon diseases with diverse pathophysiological and clinical features. Their occurrence in the same patient is highly unusual and interesting. We describe the case of a 32-year-old female with UC who developed TAK, 4 years after the initial diagnosis. She was also found to have the HLA subtype B52 which has been commonly reported in these two conditions. We discuss the similarities and differences in clinico-epidemiological, pathophysiological, and genetic features of TAK and UC. We also performed a literature search and reviewed similar case reports and case series of co-occurrence of TAK and UC, between 2010 and 2021.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic autoimmune disorder with a variety of manifestations. Association with other systemic autoimmune diseases is uncommonly reported in the literature. Hence, we intended to understand better the uniqueness of the overlapping associations of IgG4RD with other systemic autoimmune diseases. Retrospective electronic medical records-based review of IgG4RD occurring as an overlap with other systemic autoimmune diseases seen in the rheumatology department was done. We present two cases of IgG4 disease overlapping with systemic rheumatic diseases with a brief review of the literature. The first case was an overlap IgG4RD with lupus and the second interesting case had IgG4RD overlapping with 2 autoimmune diseases (lupus and rheumatoid arthritis) in the same patient. Both patients were treated with high-dose steroids and mycophenolate mofetil with good response, however, the second patient needed to be additionally treated with rituximab for better control of arthritis. Although uncommon, here, we report the first case series of 2 patients with IgG4 disease overlapping with other systemic autoimmune diseases. In our case series, the presence of the overlap did not impact the immediate clinical outcome or treatment response of IgG4RD or the co-existent systemic autoimmune disease.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis predominantly affecting medium-sized muscular arteries. PAN is comparatively rarer in childhood. It can present either in the form of cutaneous lesions (c-PAN) or systemic involvement (classic PAN). Classic PAN involves multiorgan system whereas cutaneous PAN has benign, chronic and relapsing course characterized by involvement of skin, muscles, and joints. PAN presenting as gangrene of peripheral limbs is a rare presentation. Very few cases have been reported in the Indian Pediatrics literature. Here, we report a case of peripheral limb gangrene presenting as the initial manifestation of PAN.

Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse drug reaction caused by drugs in the majority of cases. It is characterized by the sudden onset of nonfollicular sterile pustules, papules, and edematous plaques on an erythematous background, predominantly involving the flexures. Sulfa drugs have a notorious reputation of wide range of severe adverse reactions, including AGEP. However, nonantibiotic sulfonamides behave differently from antibiotic sulfonamides in the pattern of adverse drug reactions and seldom cause AGEP. Only one case of AGEP due to salazosulfapyridine has been documented, none due to sulfasalazine. We report a 40-year-old female patient with a known case of reactive arthritis who was started on tablet sulfasalazine 500 mg in escalating doses, 4 days before the onset of nonfollicular sterile pustules, on erythematous background predominantly over flexures. She was diagnosed as a case of definite AGEP with score of 12/12 on the European Study of Severe Cutaneous Adverse Reaction validation scale. As per the Naranjo causality scale, sulfasalazine was the probable drug with a score of 5.