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Aim: Antineutrophil cytoplasmic antibody‐associated vasculitides (AAV) are a group of small vessel vasculitis with systemic presentations and considerable morbidity and mortality. Pregnancy in these patients poses a significant therapeutic challenge. There is limited published literature regarding pregnancy in AAV. Methods: Two cases of successful pregnancy outcomes in patients with active AAV are described. A systematic review was conducted on the lines of the PRISMA statement for conducting systemic reviews: PubMed (inception of PubMed until 30 April 2017, English language only) and EmBase databases were searched using the following terms: 'pregnancy' AND 'ANCA associated vasculitis' OR 'granulomatosis with polyangiitis' OR 'eosinophilic granulomatosis with polyangiitis' OR 'microscopic polyangiitis' OR 'Churg‐Strauss syndrome' OR 'Wegener's granulomatosis'. Results: One hundred and thirty‐seven pregnancies were documented in 110 patients of AAV. Vasculitis diagnosis was made before pregnancy in 69, during pregnancy in 32 and after pregnancy in 9 patients. Mean age at the time of pregnancy was 29.3 ± 5.3 years. There were 91 term pregnancies, 28 were preterm pregnancies, 15 abortions and 3 still births; 78 had normal delivery and 26 had caesarian section. Conclusion: Successful pregnancies have been reported in AAV patients. [ABSTRACT FROM AUTHOR]

The objective of the study is to report the outcomes of COVID-19 in ANCA-associated vasculitis (AAV) patients. This was a registry-based observational study conducted at a tertiary care center in north India. AAV patients with at least one follow-up visit between March 2020 and September 2021 were included. Demographic features, clinical manifestations, disease activity, and treatment details of underlying AAV were noted in all patients. Details of COVID-19 infection including severity, treatment, and outcomes were noted. Predictors of COVID-19 severity were determined using univariate analysis. A total of 33 (18.3%) out of 180 AAV patients contracted COVID-19 infection. Moderate COVID-19 infection was seen in 33.3% and severe or critical infection was seen in 36.3% of patients. Seventeen patients (51.5%) required supplemental oxygen therapy. Nine patients had active disease at the time of COVID-19 infection and three of them died due to COVID-19 infection. The risk of COVID-19 infection and its severity did not differ between patients receiving different immunosuppressants including rituximab induction. Hypothyroidism (p = 0.046) and ocular (p = 0.038) involvement due to AAV predicted the development of moderate to severe/critical COVID-19. Three (9.1%) patients died from COVID-19 and the rate of AAV flare after COVID-19 was similar to that in non-COVID-19 patients (15.3/100 person-year vs. 15.6/100 person-year, p = 0.95). Majority of the patients with AAV had moderate to severe or critical COVID-19 infection. The rate of death due to COVID-19 in AAV is higher than in general population. Use of standard remission induction regimens did not lead to increased risk of COVID-19 infection in our AAV cohort. [ABSTRACT FROM AUTHOR]

Background: Unlike adults, primary membranous nephropathy (PMN) comprises only 1–2% of childhood nephrotic syndrome. The clinical behaviour of PMN in children is not explicit and we report upon clinical presentation and outcome. Methods: This prospective study includes children and adolescents (< 20 years) with biopsy-proven PMN without secondary causes. Anti-PLA2R assessment: before and after completing therapy. Outcome: percentage of patients achieving remission. Results: Study cohort included 48 (M:F ratio 1.1:1) patients and median age 17 (IQR 15–18) years, with 35 (72.9%) PLA2R related. Median interval from symptom onset to presentation was 5 months, where median proteinuria, serum albumin and creatinine were 4.9 g/day, 2.1 g/dL and 0.63 mg/dL, respectively. Forty-seven patients received immunosuppressive therapy, with various agents used as first-line therapy: cyclical CYC/GC (53.1%), CNI/GC (21.3%), rituximab (14.9%), prednisolone alone (4.3%), azathioprine (4.3%) and mycophenolate mofetil (2.1%). Median follow-up was 29 (14, 59) months. At 6 months, 11 (24.4%) and 17 (37.7%) had complete remission (CR) or partial remission (PR), while at last follow-up (median 29 months), 20 (45.4%) and 14 (31.8%) had CR and PR respectively. No significant differences in outcome were observed with different agents. A total of 60% patients treated with rituximab as first line/for relapsing disease, and all cases with resistant disease receiving rituximab had CR or PR at last follow-up. PLA2R antibody presence was associated with clinical outcome. Conclusions: Three-quarters of PMN in children and adolescents is PLA2R related and two-thirds respond to immunosuppressive therapy. Rituximab is a promising agent to manage PMN in children. Anti-PLA2R is associated with clinical outcomes. [ABSTRACT FROM AUTHOR]

Background: Collapsing focal segmental glomerulosclerosis (cFSGS) is an aggressive glomerular disease presenting as a nephrotic syndrome that has lower rates of remission with conventional immunosuppressive therapy and rapidly progresses to end‐stage‐renal‐disease (ESRD). We report eight cases of HIV‐negative cFSGS treated with rituximab. Methods: The current report is a retrospective case series of cFSGS treated with rituximab from January 2011 to March 2020, at varying phases of the disease. Results: Eight out of the 70 cFSGS patients received rituximab. The median age of patients was 30 years (IQR 24.25‐37.5); five patients were males. The median serum creatinine, mean serum albumin and median 24 hours urinary protein at presentation was 0.9 (IQR 0.66‐1.27) mg/dL, 2.95 ± 1.15 g/dL, 4.87 (IQR 1.64‐5.75) g/day, respectively. Two patients were steroid‐resistant, one steroid and tacrolimus dependent, one steroid and cyclosporine dependent, two steroids and tacrolimus resistant, one steroid, tacrolimus, cyclophosphamide, mycophenolate mofetil resistant and one steroid‐resistant and tacrolimus dependent before rituximab therapy. Rituximab was given either as targeted therapy (after an initial dose of 375 mg/m2; patients having CD‐19 levels >5/μL or >1% at 1 month received additional low‐dose [100 mg] of rituximab), or weekly regimen. Five patients received CD‐19 targeted rituximab; three received weekly doses of 375 mg/m2, cumulative doses being 820 ± 228.03 mg, and 1800 ± 721.11 mg, respectively. At the end of median follow‐up of 15 months, five (62.5%) patients were in remission (three partial, two complete remissions), two (25%) were resistant to therapy; one (12.5%) progressed to ESRD. Conclusion: Rituximab is reasonably safe and achieves/maintains remission in 60% of cFSGS cases. SUMMARY AT A GLANCE: This is a retrospective study reporting the effects of Rituximab on 8 patients with the collapsing form of focal segmental glomerulosclerosis. Whilst retrospective in nature, the study suggested that rituximab might be a promising form of treatment for this condition, which is traditionally resistant to immunosuppression therapy, resulting in a remission rate of 60% with a reasonable safety profile. [ABSTRACT FROM AUTHOR]

Objectives: To present single centre experience on the efficacy and safety of similar biologic of rituximab in patients with granulomatosis with polyangiitis (GPA). Methods: This was a retrospective study of GPA patients who received similar biologic of rituximab as either remission induction or maintenance agent. Demographic parameters, Birmingham Vasculitis Activity Score (BVAS-v3), vasculitis damage index, relapse and adverse events were retrieved from patient records. Outcomes noted were remission at 6 months in remission induction group and rates of relapses, adverse events, serious infections and mortality in both remission induction and maintenance groups. Results: Seventy-seven GPA patients were enrolled. Sixty received rituximab for induction and 57 for maintenance; 69% were anti PR-3 positive. In the induction group, median BVAS-v3 reduced from 12 (IQR 6–21.5) to 0 (0–1) at 6 months. At 6 months, 60% patients attained remission, 40% in primary induction group and 74% in re-induction group (p = 0.016%). In the maintenance group, seven (12%) patients had relapses with median time to relapse of 12 (6–22) months. Median relapse free survival was 21 (6–22) months on rituximab maintenance. There were 12 deaths (15.6%) and 18 serious infections. Conclusion: Similar biologic of rituximab was an effective agent for remission induction and remission maintenance in patients with GPA. Head to head trials with innovator molecule are needed to confirm these results. Key Points: • Remission was achieved in 60% of GPA patients who received similar biologic of rituximab as remission induction therapy. • Relapse rate during maintenance phase was 12% with similar biologic of rituximab. Serious infections and mortality with similar biologic of rituximab were comparable with that reported previously in AAV trials. [ABSTRACT FROM AUTHOR]