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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.

BackgroundSLE is characterised by excessive production of various autoantibodies and correlation of these antibodies with organ involvement may help to evaluate disease severity and long term prognosis. NBTE is a rare cardiac manifestation of SLE with prevalence rate varying from 6%–11%. Many, but no all, studies have shown association of NBTE with anti phospholipid antibodies, but, except this association, data regarding clinical, laboratory and serological characteristics of NBTE is sketchy. We designed this study to evauate profile of patients having NBTE in SLE.Objectives1. To study the prevalenence of NBTE in SLE patients.2. To study association of NBTE with clinical and laboratory characteristics and serological profile.MethodsAll consecutive SLE inpatients and outpatients attending the department of Rheumatology from September 2015 to December 2017 were enrolled. Patients subjected to 2D Echo were included and their demographic, clinical, laboratory and serological profile were recorded. Serological profile was studied with Blue diver kit which is an immunodot blot assay measuring autoantibodies against 25 ENA. Anti cardiolipin and anti beta 2 glycoprotein antibody were tested by ELISA. Study was approved by an independent ethics committee [ECR/282].ResultsTotal number of patients enrolled in study were 355 out of which 213 had undergone 2DEcho. NBTE was found in 33 (15.49%) patients. Among all autoantibodies studied, we found that the presence of anti-Nucleosome antibody, LAC, ACL and B2GP1 were significantly associated with NBTE (p<0.05). Myocarditis, valvular lesions and Pulmonary Hypertension were more common in NBTE group (p value: 0.012,<0.0001 and 0.013 respectively).We also noticed that there is a statistically significant association between presence of NBTE with APLA syndrome and Thrombotic events (p value<0.0001 and 0.005 respectively).Tab.1 Significant Serological association of SLE patients with NBTE.AntibodiesSLE With NBTE-33 (15.49%)SLE without NBTE-180P value Anti-nucleosome27 (81.8)32 (17.7)<0.0001LAC16/30 (53.3)31/149 (20.8)0.0002ACL (Ig M and IgG)9/303013/128 (10.1)0.004B2GP-1(Ig M and IgG)6/22209/103 (8.73)0.033APLA profile was available in 30 patients of NBTE and 147 patients not having NBTE. Out of this, positivity for APLA antibodies were seen in 17 (56.6%) and 36 (24.4%) patients respectively [p:0.005]. 82.3% patients with Anti phospholipid antibodies had APLA syndrome in NBTE group while in NBTE group 48.5% patients having Anti phospholipid antibodies had APLA syndrome. Thus, presence of NBTE increased the possibility of developing APLA syndrome in patients having positive serology for anti phospholipid antibodiesTab.2 Significant Clinical and laboratory characteristic of SLE patients with NBTE.Organ involvementSLE with NBTE-33SLE without NBTE-180p- value Myocarditis11270.012Valvulopathy104<0.0001PAH9210.013Thrombotic events8160.005APLA syndrome1417<0.0001ConclusionsPresence of Anti nucleosome antibody, LAC, Anti cardiolipin and anti beta 2 glycoprotein antibodies may predict presen...

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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 patients (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 involvement 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.