H01688 | |
H number | H01688 |
Name | Rapidly progressive glomerulonephritis |
Description | Rapidly progressive glomerulonephritis (RPGN) or crescentic glomerulonephritis is a life-threatening disease that destroys kidneys over a period of days to weeks. Proliferation of epithelial cells and infiltration of inflammatory cells lead to glomerular crescent formation and disruption of the specialized microvascular network in the glomerulus. This causes hematuria, albuminuria and loss of renal function. From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-glomerular basement membrane antibody (anti-GBM) GN, and immune complex GN. PIGN, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence or electron microscopy. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA). Corticosteroids and cyclophosphamide are the first-line treatment, but infection is the most common cause of death of RPGN patients. Several new treatment strategies, such as leukocytaphereis (LCAP) and intravenous immunoglobulin (IVIg), have become available and these treatments have made it possible to treat high-risk RPGN patients without inducing serious immunosuppressive states. |
Category | Urinary system disease |
Network | - |
Gene | - |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | - |
Comment | See also H01655 Wegener's granulomatosis |
Other DBs | ICD-11: GB40 ICD-10: N01.9 MeSH: C538458 |
Reference | PMID:26798468 AUTHORS Cheungpasitporn W, Zacharek CC, Fervenza FC, Cornell LD, Sethi S, Herrera Hernandez LP, Nasr SH, Alexander MP TITLE Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. JOURNAL Clin Kidney J 9:97-101 (2016) DOI:10.1093/ckj/sfv126 PMID:21946538 AUTHORS Bollee G, Flamant M, Schordan S, Fligny C, Rumpel E, Milon M, Schordan E, Sabaa N, Vandermeersch S, Galaup A, Rodenas A, Casal I, Sunnarborg SW, Salant DJ, Kopp JB, Threadgill DW, Quaggin SE, Dussaule JC, Germain S, Mesnard L, Endlich K, Boucheix C, Belenfant X, Callard P, Endlich N, Tharaux PL TITLE Epidermal growth factor receptor promotes glomerular injury and renal failure in rapidly progressive crescentic glomerulonephritis. JOURNAL Nat Med 17:1242-50 (2011) DOI:10.1038/nm.2491 |