Abstract
Background: The spectrum and outcomes of crescentic glomerulonephritis (Cr.GN) in South Asia is vastly different from that reported worldwide and there is a paucity of information. The aim of the study was to study the demography, clinical presentation, histology and predictors of longitudinal outcomes of Cr.GN in this population. Methods: An observational cohort study of renal biopsies was performed in the largest tertiary center in South India over a period of 10 years (January 2006 to December 2015) with ≥50% crescents on renal histology indicating Cr.GN. Results: A total of 8645 kidney biopsies were done; 200 (2.31%) were Cr.GN. Patients were categorized into three etiological groups: anti-glomerular basement membrane (type I), immune complex (type II), and pauci-immune (type III). Type II was the most common (96, 46.5%), followed by type III (73, 38%) and type I (31, 15.5%). Female preponderance was seen across all types. About half of all patients presented with recent onset hypertension. Type II had the highest median proteinuria (4.2 (2.1-6) g/day, p=0.06) and the median estimated glomerular filtration rate was lowest in type I (5 (4-8) ml/min/1.73m 2, p<0.001). Among type III, anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis was seen only in ~50% of patients. Nearly one third of patients with type I were also positive for ANCA making them 'double positive'. Acute glomerular insults like tuft necrosis and chronic changes as evidenced by moderate to severe interstitial fibrosis, was a predominant feature of type I. Conclusions: ANCA-negative pauci-immune vasculitis, as well as double positive Cr.GN, are reported for the first time in South-Asia. Renal survival was significantly worse in type I/III compared to type II. Types I/III, moderate to severe interstitial fibrosis and tubular atrophy, presence of oliguria/anuria and increasing percentage of crescents in renal biopsy were significant predictors of end stage kidney disease in our cohort.
Highlights
Crescentic glomerulonephritis (Cr.GN) is defined histologically by the presence of extensive glomerular crescents
It is known as rapidly progressive glomerulonephritis (RPGN) as it is accompanied by rapid decline in renal functions
The mean age of presentation for all types was 40.6±14.6 years, with the highest mean age of presentation seen in patients with type III Cr.GN
Summary
Crescentic glomerulonephritis (Cr.GN) is defined histologically by the presence of extensive glomerular crescents (usually greater than 50%) It is known as rapidly progressive glomerulonephritis (RPGN) as it is accompanied by rapid decline in renal functions. It can occur in any glomerular disease[1] and is usually reported in about 4% to 10% of native kidney biopsies[2]. Pathogenesis of glomerular diseases involves a complex and as yet incompletely understood interplay between epigenetic, immunoregulatory, hormonal, and environmental factors on a background of genetic predisposition[8] This translates into a broad spectrum of disease presentation, a variable tempo of progression and heterogenous outcomes, which are evident from these previous studies. Methods: An observational cohort study of renal biopsies was performed in the largest tertiary center in South India over a period of 10 years (January 2006 to December 2015) with ≥50% crescents on renal histology indicating Cr.GN
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
