Etiological Spectrum and Clinical Features in 215 Patients of Crescentic Glomerulonephritis: Is it Different in India?

Indian J Nephrol. 2021 Mar-Apr;31(2):157-162. doi: 10.4103/ijn.IJN_237_19. Epub 2021 Mar 8.

Abstract

Introduction: Crescentic glomerulonephritis (CrGN) characterized by the presence of crescents in most (≥50%) glomeruli on renal histology clinically presents as rapidly progressive renal failure. It can occur due to diverse etiologies with varying course and renal outcomes. We studied the prognostic significance of its classification as pauci-immune, anti-GBM, and immune-complex mediated CrGN.

Materials and methods: Renal biopsies diagnosed as CrGN over 9 years were included. Clinical, biochemical, serological, and histological features of various classes of CrGN were correlated with renal outcome.

Results: 215 biopsies were diagnosed as CrGN during this period. A majority (63%) were immune-complex mediated while 32% were pauci-immune, followed by anti-GBM disease (5%). 85.5% of pauci-immune CrGN were ANCA associated. The levels of proteinuria and serum creatinine were significantly higher in anti-GBM CrGN as compared to the other two classes. The various histological features including Bowman's capsule rupture, peri-glomerular granulomatous reaction, fibrinoid necrosis, and vasculitis were more common in anti-GBM disease and pauci-immune CrGN. The median renal survival was 6.3, 5.3, 2.1 months in immune-complex mediated, pauci-immune, and anti-GBM CrGN, respectively.

Conclusion: Immune-complex mediated is the commonest etiology of CrGN in India. Anti-GBM disease has the worst prognosis followed by pauci-immune and immune-complex mediated CrGN. Raised serum creatinine levels (>5mg%) and the degree of glomerulosclerosis at diagnosis were predictors of poor renal survival. High index of suspicion and prompt diagnosis can improve the outcome in these patients.

Keywords: ANCA-associated vasculitis; anti-GBM disease; crescents; glomerulonephritis.