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Introduction: To study the clinical characteristics and treatment outcomes of patients with anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM). Methods: This was a retrospective study done between 2019 and 2021 in a tertiary care center in South India. All consecutive patients, presenting to the adult rheumatology department, classified as idiopathic inflammatory myositis (IIM), and positive for anti-MDA5 antibodies, 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. Data were analyzed using the Statistical Package for the Social Sciences (SPSS) version 25. Results: A total of 29 adult patients of IIM were positive for anti-MDA5 antibody during the study period. The mean (±standard deviation) age of the patients was 40.3 (±13.02) years with a female:male ratio of 1.4:1. Panniculitis, calcinosis, palmar papules, and ulcerated Gottron's were the specific cutaneous manifestations, seen in 3 (10%), 7 (24%), 4 (13.4%), and 7 (24%) patients, respectively. A total of 14 patients (48.1%) had clinically amyopathic DM and 17 patients (60.7%) had interstitial lung disease (ILD) diagnosed by high-resolution computerized tomography scan, of which organizing pneumonia was the predominant pattern. Complete response was seen in 10 patients (43.4%), while a partial response was seen in 8 patients (34.7%). Five patients died on follow-up, accounting for mortality of 21%. Age >50 years was significantly associated with mortality (P = 0.025). 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-MDA5 IIM group, whereas myopathy (P < 0.001), elevated creatine phosphokinase (P < 0.001), and lactate dehydrogenase (P < 0.001) were more frequently present in the non-anti-MDA5 IIM group (n = 421). Conclusions: Anti-MDA5 DM represents a distinct and unique subset of IIM with characteristic clinical manifestations. Elderly age is a poor prognostic factor of mortality.

Statin-naïve immune-mediated necrotizing myopathy (IMNM) is a rare disease entity, the diagnosis of which is complicated in the absence of a reliable biomarker. In this context, this case, which is the first one reported from a tertiary care center in South India, is unique. The presence of hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) antibody supplements the clinical diagnosis, specifically in the serological absence of myositis-specific antibodies. A comprehensive diagnostic approach including HMGCR antibody positivity is advisable in IMNM.

Background: Systemic lupus erythematosus (SLE), a rare multisystem disorder with a female preponderance, has a high cost for the care; however, there is no evidence relevant to the Indian setting. The primary objective of our study was to determine the financial burden of the index admission (IA) and subsequent costs during follow-up and ascertain the proportion with a catastrophic health expenditure (CHE). Methodology: This was an observational retrospective cohort study where inpatients of a general medicine ward were recruited from January 2019 to October 2020. Clinical details and costs were obtained from the hospital's electronic records and bills. Patients were telephonically interviewed for follow-up clinical details and costs incurred. A patient-family payer perspective was used. Linear regression analysis was used. Results: Of the 73 patients recruited during the study period, 96% were females and the majority (71%) were admitted through casualty, with 59% of patients having high disease activity (SLE Disease Activity Index >12). The hospital mortality was 9.6%. After a median follow-up of 12 months, there was good quality of life with no difference between the two severity groups. The total cost of the IA was 135,768 INR (94,053–223,954) and it was higher for the severe disease group (P = 0.038). The direct medical costs compromised 83% of admission costs. In the multivariate regression, the duration of hospital and intensive care unit stay were predictors of high cost. The median 6 months follow-up cost was 32,978 (14,240–80,940) and the total calculated annualized cost was 202,124 (136,188–331,508), which was not statistically different between the two groups. There was a CHE among 86% of patient-families. Conclusion: This study demonstrates that there is high morbidity and cost involved in the management of a flare of SLE. However, with appropriate care, there are reasonably good outcomes and quality of life beyond six months.

Introduction: Safety of rituximab (RTX) in autoimmune rheumatic diseases (AIRDs) has been a concern during this ongoing coronavirus (COVID-19) pandemic. Delayed worsening of COVID symptoms, increased hospital stays, and mortality has been reported among those infected post-RTX. This study describes the occurrence and course of COVID-19 infection among AIRD Indian patients who received RTX during this pandemic. Patients and Methods: Adult patients (≥18 years) with any AIRD, who received RTX between October 2019 and May 2021, were enrolled in this study. Patients satisfying the inclusion criteria were telephonically enquired about contracting confirmed COVID-19, its course, treatment, and outcome. Baseline parameters, immunoglobulin G level, vaccination, and comorbidity status before RTX were compared between the COVID-19 infected and noninfected patients to determine factors affecting the outcome. Results: Out of 1081 patients admitted during the study period, 218 patients received RTX. The mean age of these patients was 40.1 ± 14.2 years and comprised mostly of women (81.7%). Rheumatoid arthritis followed by lupus and anti-neutrophilic cytoplasmic antibodies-associated vasculitis was the predominant AIRD. Among the patients contacted (207/218 [94.9%]), 11 (5.3%) patients reported confirmed COVID-19 infections. Out of these, three (27.3%) had severe COVID-19 and one patient succumbed to it. Others became symptom-free after a mean duration of 14.4 ± 4.7 days of onset of symptoms. No significant difference among the baseline parameters observed predicted COVID-19 susceptibility. Conclusion: Among our AIRD patients treated with RTX, occurrence, and mortality of COVID-19 infection was low. Younger age of our patient cohort and female predominance might have contributed in reducing the severity of SARS-CoV-2 infection.

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.

Sarcoidosis is a multisystem inflammatory disorder, which can have a myriad of presentations, with atypical presentation not being uncommon. Unless the clinician has a strong suspicion of sarcoidosis, it can be easily missed out. Here, we present an interesting case of a 44-year-old gentleman who had presented with inflammatory arthritis and vomiting. His serum cortisol levels were low. On further evaluation, he was found to have panhypopituitarism. Magnetic resonance imaging brain showed infiltrative disease involving the pituitary stalk. Imaging of the chest revealed mediastinal nodes which showed nonnecrotizing granulomatous inflammation on histopathology. After ruling out infectious causes a diagnosis of sarcoidosis was made, and the patient had a full recovery with steroids and methotrexate. Sarcoidosis can be a great mimicker presenting with a myriad of complications. Clinicians must be aware of the atypical presentations to diagnose the disease earlier.

Leprosy and sarcoidosis are chronic multisystem disorders, with clinical features overlapping with each other. The diagnosis requires a thorough clinical, laboratory, histopathological, and radiological examination. Although it is possible to conclude on one particular diagnosis most of the time, in some cases, even after extensive evaluation, it is difficult to differentiate between the two diseases. In such scenarios, treating both the conditions is warranted. Here, we present a case with a diagnostic dilemma between Hansen's disease and sarcoidosis and systematically discuss the specific pointers which can help to differentiate between the two diseases.

Background: We aimed to evaluate the high-resolution ultrasonography (HRUS) findings in the hands of patients with psoriatic arthritis (PsA) and rheumatoid arthritis (RA). Materials and Methods: This was a cross-sectional study of ultrasonography (USG) of hands in patients with PsA fulfilling the Classification criteria for psoriatic arthritis (CASPAR) in comparison with cases with RA fulfilling 1987 American College of Rheumatology classification criteria. Bilateral 1st and 2nd metacarpophalangeal (MCP) and proximal interphalangeal joints were assessed for synovitis and bone erosions, and extensor tendons at wrist for tenosynovitis by a single observer using the GE LOGIQ BOOK XP USG machine (General Electric Medical Systems) with a high-frequency 12-MHz linear array transducer. Demographic and clinical details were extracted from the electronic database of the hospital information system. Results: A total of 62 patients (31 with RA and PsA each) were studied. Demographic and baseline features were similar, except for more male patients in PsA as compared to RA (41.9% vs. 16.1%; P = 0.02). MCP synovitis was higher in RA as compared to PsA (71% vs. 29%; P = 0.001). Multiple logistic regression analysis found that absence of MCP synovitis by HRUS (adjusted odds ratio [OR]: 5.97; P = 0.02) in spite of higher number of swollen joints (adjusted OR: 29.3; P = 0.03) favors the diagnosis of PsA as compared to RA. Conclusion: “Our finding of fewer or no MCP synovitis by HRUS in PsA as compared to RA warrants a larger, powered validation study; if reproduced, it can be an additional tool to differentiate PsA including those without overt skin lesions from seronegative RA/early undifferentiated polyarthritis.”

Objectives: The objective of this study is to study the role of urinary vascular cell adhesion molecule-1 (uVCAM-1) as a marker for disease activity in lupus nephritis (LN). Methodology: This was a diagnostic study where patients with active LN were taken as cases and those without LN were taken as disease controls. uVCAM-1 was correlated with the classes of LN and standard of care markers. Results: There was a significant difference between the uVCAM-1 values in cases (59.69 [range: 0.07–13752.5]) pg/mg and controls (2.11 [range − 0.11 − 1138.5] pg/mg [P = 0.02]). Maximum levels of VCAM 1 were seen in Class 4 and Class 5 LN (P = 0.96). Although higher renal systemic lupus erythematosus disease activity index score had a higher median value of uVCAM 1, the values were not statistically significant (P = 0.2). There is a positive correlation between the uVCAM1 and anti-double-stranded DNA (anti-dsDNA) levels (r = 0.38) and a negative correlation between VCAM 1 levels and C3 (r = −0.19).The sensitivity of uVCAM1 for determining the disease activity was 65.2%, and the specificity was 75% at a cutoff value of more than 23.8 pg/mg. Conclusions: uVCAM 1, although not in isolation, but along with the other standard of care markers may be useful in assessing the disease activity.

A 51-year-old female with mixed connective tissue disease presented with bilateral sensorineural hearing loss. The hearing deficit was gradually progressive over a period of 6 months. On evaluation, no obvious cause for hearing was evident. Due to ototoxic potential of hydroxychloroquine, we decided to stop the drug and observe for improvement. Over time, her hearing loss stabilized, with improvement in audiometric findings. Hydroxychloroquine induced auditory toxicity is rare. Increased awareness and early recognition may minimize damage.