Rapidly progressive glomerulonephritides (crescentic glomerulonephritides)

Czech version

Authors: O. Zakiyanov ^1,2; J. Vachek ^1,3; V. Tesař ¹
Authors‘ workplace: Klinika nefrologie VFN a 1. LF UK v Praze ¹; Ústav lékařské biochemie a laboratorní diagnostiky VFN a 1. LF UK v Praze ²; Farmakologický ústav 1. LF UK v Praze ³
Published in: Kardiol Rev Int Med 2014, 16(1): 62-66
Category: Internal Medicine

Overview

Rapidly progressive glomerulonephritides (RPGN) are the most severe form of the nephritic syndromes. In this case, glomerular injury is so acute that irreversible renal failure develops over the course of a few days to weeks. RPGN usually presents as proteinuria < 3g/ day, hematuria with dysmorphic red blood cells and/ or red cell casts, with or without signs of systemic vasculitis. A specific finding upon kidney biopsy is a crescent formation in more than 70% of glomeruli. The most common diseases presenting as RPGN are ANCA‑associated vasculitides, Goodpasture’s disease (anti‑renal nephritis), and the much less common immunocomplex glomerulonephritides. Treatment includes glucocorticoids, cyclophosphamide and novel therapies including targeted treatment (rituximab), sometimes in combination with plasma exchange.

Keywords:
rapidly progressive glomerulonephriti – pulmorenal syndrome – crescents – renal failure – cyclophosphamide – plasma exchange

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