Anti-glomerular basement membrane disease: Case series from a tertiary center in North India

Abstract

Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months. All the patients were treated with steroids, cyclophosphamide, and plasma exchange. A total of 17 patients (nine males) were included. The mean age at presentation was 39.11 ± 16.58 (range 11–72) years. Twelve patients (70%) presented with rapidly progressive glomerulonephritis (RPGN), 4 (23.5%) presented with Goodpasture syndrome, while 1 (5.8%) had nephritic syndrome, 7 (41%) were hypertensive, and 14 (82.3%) required dialysis at the time of presentation. Four patients (23.5%) had associated anti-neutrophil cytoplasmic antibody positivity (anti-myeloperoxidase antibodies in all). Fourteen (87.5%) patients had crescentic glomerulonephritis, while 5 (31.25%) showed necrotizing (n = 4) or granulomatous (n = 1) in the vasculitis. Of 16 patients who received treatment, four (23.25%) achieved complete remission. In this single-center study, the majority of anti-GBM disease patients presented with RPGN and had crescentic glomerulonephritis on biopsy with poor treatment outcome.

Clinical Profile and Renal Survival of Anti-Glomerular Basement Membrane Disease Patients: A Retrospective Case Series from Northern India

“…The possible reasons could be because of better healthcare facilities in terms of manpower and access in their center compared to our setting, which is a remote, hilly terrain with low resources. The one-year renal survival in Indian studies was 23% and 9%, whereas in our study it was 26% [4,12]. Studies done in Europe had shown one-year renal survival and overall patient survival of 32-44% and ~87% respectively, much higher as compared to all Indian studies [12,23].…”

“…In our study, the most common presenting complaints were nonspecific prodromal symptoms in 80% of the patients, while renal-specific symptoms such as anuria were seen in 2 patients and hematuria in one. This may be [4,12,13]. In the present study, the age of presentation was in between European and Indian studies; maybe we are missing the older population.…”

Temporal Clustering of Antiglomerular Basement Membrane Disease in COVID-19 Pandemic: A Case Series

“…Most patients were affected in their third to the fifth decade. 5 It is observed from more extensive case series that anti-GBM has bimodal age distribution with peak incidence during the third decade and presentation with pulmonary renal disease and in the sixth and seventh decades, where presentation with isolated kidney involvement is more common. 6 There are no case reports of anti-GBM disease associated with COVID-19 in India.…”

Therapeutic plasma exchange in renal disorders

“…[2223] A prospective study from North India found that most patients presented with dialysis-dependent renal failure with poor prognosis. [24] A Chinese center found that serum level of anti-GBM antibodies was an independent factor associated with mortality. [25] Early implementation of TPE targeting undetectable levels of anti-GBM antibodies is the goal of the therapy.…”

“…Patients who were dialysis dependent and having 100% crescents did not recover renal function despite therapy. [24] However, even in patients presenting with renal failure requiring dialysis, it is appropriate to initiate adjunct TPE. Renal biopsy done at the earliest opportunity showing 100% crescents (regardless of cellularity – personal opinion) indicates that renal recovery is unlikely and disease-modifying treatment including TPE can be withdrawn.…”