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

• Published in: Indian journal of nephrology Vol. 27; no. 2; pp. 108 - 112
• Main Authors: Prabhakar, D, Rathi, M, Nada, R, Minz, RW, Kumar, V, Kohli, HS, Jha, V, Gupta, KL
• Format: Journal Article
• Language: English
• Published: India Medknow Publications and Media Pvt. Ltd 01.03.2017; Scientific Scholar; Medknow Publications & Media Pvt Ltd
• Subjects: Biopsy; Care and treatment; Case studies; Conflicts of interest; Gangrene; Glomerulonephritis; Hemodialysis; Immunoglobulins; Inflammation; Liver diseases; Medical prognosis; Original; Patients; Plasma; Tuberculosis; India; Goodpasture syndrome; rapidly progressive glomerulonephritis; crescentic glomerulonephritis; Anti-glomerular basement disease
• ISSN: 0971-4065; 1998-3662
• DOI: 10.4103/0971-4065.171227

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 ( = 4) or granulomatous ( = 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.