Anti-glomerular basement membrane disease: Case series from a tertiary center in North India
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 conduc...
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doaj-d62c554c1ea943e6bee36485ded60e7e2020-11-24T20:51:49ZengWolters Kluwer Medknow PublicationsIndian Journal of Nephrology0971-40651998-36622017-01-0127210811210.4103/0971-4065.171227Anti-glomerular basement membrane disease: Case series from a tertiary center in North IndiaD PrabhakarM RathiR NadaR W MinzV KumarH S KohliV JhaK L GuptaAnti-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.http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2017;volume=27;issue=2;spage=108;epage=112;aulast=PrabhakarAnti-glomerular basement diseasecrescentic glomerulonephritisGoodpasture syndromerapidly progressive glomerulonephritis |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
D Prabhakar M Rathi R Nada R W Minz V Kumar H S Kohli V Jha K L Gupta |
spellingShingle |
D Prabhakar M Rathi R Nada R W Minz V Kumar H S Kohli V Jha K L Gupta Anti-glomerular basement membrane disease: Case series from a tertiary center in North India Indian Journal of Nephrology Anti-glomerular basement disease crescentic glomerulonephritis Goodpasture syndrome rapidly progressive glomerulonephritis |
author_facet |
D Prabhakar M Rathi R Nada R W Minz V Kumar H S Kohli V Jha K L Gupta |
author_sort |
D Prabhakar |
title |
Anti-glomerular basement membrane disease: Case series from a tertiary center in North India |
title_short |
Anti-glomerular basement membrane disease: Case series from a tertiary center in North India |
title_full |
Anti-glomerular basement membrane disease: Case series from a tertiary center in North India |
title_fullStr |
Anti-glomerular basement membrane disease: Case series from a tertiary center in North India |
title_full_unstemmed |
Anti-glomerular basement membrane disease: Case series from a tertiary center in North India |
title_sort |
anti-glomerular basement membrane disease: case series from a tertiary center in north india |
publisher |
Wolters Kluwer Medknow Publications |
series |
Indian Journal of Nephrology |
issn |
0971-4065 1998-3662 |
publishDate |
2017-01-01 |
description |
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. |
topic |
Anti-glomerular basement disease crescentic glomerulonephritis Goodpasture syndrome rapidly progressive glomerulonephritis |
url |
http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2017;volume=27;issue=2;spage=108;epage=112;aulast=Prabhakar |
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