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Objectives: This cross-sectional study was designed to assess the clinical profile and frequency of associated autoantibodies in all consecutive patients classified as systemic sclerosis (SSc) at Medanta—the Medicity Hospital, Gurgaon, India. Methods: Between August 2017 and July 2019, we identified a total of 119 consecutive patients meeting the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2013 criteria for SSc and 106 patients consented to this study. Their clinical and serological data at the time of enrolment were analysed. Results: Our cohort had a mean age at symptom onset of 40 ± 13 years with a median symptom duration of 6 years. We had 76 patients (71.7%) with interstitial lung disease (ILD), which was a higher proportion compared to European cohorts. 62 pa- tients (58.5%) had diffuse cutaneous involvement which was significantly associated with anti-Scl70 antibodies (p < 0.001), digital ulcers (p = 0.039) and the presence of ILD (p = 0.004). 65 patients (61.3%) had anti-Scl70 and 15 patients (14.2%) had anti-centromere (anti-CENP) antibodies. Scl70 positivity was associated with the presence of ILD (p < 0.001) and digital ulcers (p = 0.01). Centromere antibodies had a negative association with ILD (p < 0.001), but was a risk factor for calcinosis (p < 0.001) and pulmonary arterial hypertension (PAH) (p = 0.01). The combination of diffuse cutaneous disease and Scl70 antibodies was the strongest predictor of ILD and digital ulcers (p = 0.015). sm/RMP, RNP68 and Ku antibodies correlated with musculoskeletal involvement (p < 0.01), while all seven of the patients with Pm/Scl antibodies had ILD. Renal involve- ment was noted in only two patients. Limitations: A single-centre study may not capture the true prevalence of disease characteristics in the population. Referral bias for patients with diffuse cutaneous disease has been noted. Data on RNA-Polymerase antibodies have not been provided. Conclusion: North Indian patients have some characteristic differences in disease phenotype as compared to their Caucasian counterparts with a larger proportion of patients presenting with ILD and Scl70 antibodies. Antibodies against Ku, RNP and Pm/Scl occur in a minority of patients, but may be associated with musculoskeletal features. [ABSTRACT FROM AUTHOR]

Background: Induction with cyclophosphamide (CYC) and glucocorticoids in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) has a relapse of 30%–50%. Studies show that rituximab (RTX) is superior to CYC in refractory/relapsed AAV. We prospectively analyzed efficacy and safety of RTX in CYC-failed cases of AAV. Methods: Patients with AAV who relapsed or were refractory to CYC therapy were given RTX 1 gm at 0 and 15 days, followed by maintenance with 500 mg every 4–6 months. All patients received oral prednisolone. Disease activity was defined by Birmingham Vasculitis Activity Score/Wegener's granulomatosis (BVAS/WG). Remission was defined by the European League Against Rheumatism criteria. Results: From August 2012 to July 2018, 67 patients with AAV were seen at our center; 21 patients who relapsed after inductions with CYC or were refractory to CYC received RTX; 8 (38%) were refractory and 13 (62%) were relapsed AAV; 20 were anti-proteinase 3 positive and 1 was anti-myeloperoxidase positive. All were granulomatosis with polyangiitis (GPA). Mean time to relapse was 12.04 ± 7.8 months. Most common indication for RTX was lung followed by ophthalmic, renal, ear nose throat, and nervous system involvement. Median follow-up after induction with RTX was 24 months. Mean BVAS/WG was 11.2 at baseline, 0.66 at the end of 3 months, 0.16 at the end of 6 months, and remained stable at that value at 18 months. At 24 months, 16 patients (76.19%) remained in remission. One was refractory to RTX treatment even after 2 years. Two patients died and two were still under follow-up. Conclusion: In our experience, RTX is a good induction and maintenance strategy for relapsed/refractory AAV.

BACKGROUND: Therapeutic plasma exchange (TPE) has been advocated as an adjunct to steroids and cytotoxic drugs in treating patients suffering from vasculitis and presenting with active disease, but we still have insufficient evidence on its effectiveness in improving the clinical response, especially in India. This study was planned to study the clinical outcome in severe vasculitic presentations treated with TPE as an adjunctive therapy. MATERIALS AND METHODS: A retrospective analysis of TPE procedures performed from July 2013 to July 2017 in the department of transfusion medicine at a large tertiary care hospital was done. All consecutive patients admitted with new diagnosis of systemic vasculitis presenting with active disease and severe presentations such as advanced renal failure or severe respiratory abnormalities or life-threatening vasculitis affecting the gastrointestinal tract, neurological and musculoskeletal system; who needed TPE for removal of preformed antibodies, were included in the study. RESULTS: There were a total of 31 patients in whom TPE was performed for severe systemic vasculitis; 26 adults and five pediatric. Six patients tested positive for perinuclear fluorescence, 13 for cytoplasmic fluorescence (cANCA), two for atypical antineutrophil cytoplasmic autoantibody, seven for anti-glomerular basement membrane antibodies, two for antinuclear antibodies (ANA), and one patient tested positive for ANA as well as cANCA before the augmentation of TPE. Out of 31, seven patients showed no clinical improvement and succumbed to the disease. At the end of desired number of procedures, 19 tested negative and five tested weak positive for their respective antibodies. CONCLUSION: Favorable clinical outcomes were observed with TPE in patients with antibody-positive systemic vasculitis. [ABSTRACT FROM AUTHOR]

Context: Autoantibodies have a role in the diagnosis and prognosis in Autoimmune Inflammatory Myositis (AIM). Aims: The aim of this work was to study the prevalence and clinical correlation of myositis specific and associated antibodies (MSA and MAA) in AIM. Setting and Design: This was a cross-sectional observational study. Methods and Materials: Consecutive AIM patents were divided into groups as dermatomyositis (DM), polymyositis (PM), CTD-associated myositis (CTD-M), cancer-associated myositis (CAM) and juvenile myositis (JM). Their data along with serum samples were collected after obtaining informed consent. Sera was analyzed for IgG antibodies against Jo-1, PL-7, PL-12, EJ, SRP, Mi-2, MDA-5, TIF1γ, SAE1, SAE2, NXP2 and SSA/R052kD using the microELISA technique. The institutional ethics committee approved the study. Statistical Analysis: SPSS software (version 24.0) was used. P value < 0.05 was considered statistically significant. Results: There were 48 patients (DM = 19, PM = 19, CTD-M = 5, CAM = 2, JM = 3) included. MSA were positive in 37.5% patients. Antibodies against Mi-2 were present in 6 (12.5%), Jo-1 in 5 (10.4%), 2 (4.1%) each had PL-7 and SRP antibodies. One patient (2%) each had MDA-5, NXP2 and TIf1g antibodies. Jo-1 antibody was associated with mechanic's hands and ILD. There was a significant association of rash in the Mi-2 group with none of the patients having ILD. Malignancy screening was negative in NXP2 and TIF1g antibody-positive patients. Ro52 was the most common MAA (33.3%) and was associated with mechanic's hand. Conclusion: MSA was present in almost 40% of the cohort. Anti Jo-1 antibody was associated with mechanic's hands and ILD. None of the Mi-2 patients had ILD, which may point to a protective role of this antibody for ILD. The association of newer antibodies in Indian patients needs to be further studied in larger cohorts. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related mastitis (IgG4-RM) is an uncommon entity in clinical practice which has an evolving spectrum of manifestations and presently is of high clinical interest among rheumatologists. Since its closest differential remains breast carcinoma, the importance of describing this entity is in the fact that early diagnosis, awareness and timely management can save the patient from unnecessary surgical intervention and its complications. Tumefactive lesions are considered hallmark of this disease, but rarely abscess may also be the presenting feature. In this article, we describe a 24-year-old female patient of a very unusual case of IgG4-RM presenting as recurrent breast abscess, its successful management and discussion about novel treatment strategies. [ABSTRACT FROM AUTHOR]

Competing Interests: The authors declare no conflict of interest.