[Rapidly progressive glomerulonephritis:classification, pathogenesis and clinical management]

Rapidly progressive glomerulonephritides (RPGN) belong to a heterogeneous group of inflammatory kidney diseases which are commonly associated with systemic vasculitic syndromes. Renal histology is characterized by necrotizing lesions within the glomerual tuft and extracapillary proliferation, in most cases leading rapidly to renal failure. The etiology and pathogenesis are only partly elucidated. Since irreversible renal scaring develops within days to weeks, RPGN represent a nephrological emergency necessitating urgent diagnostic evaluation and rapid institution of effective therapy. New onset nephritic sediment combined with concomitantly deteriorating excretory renal function should lead to immediate nephrological consultation. Autoimmune serology and particularly renal biopsy are of the utmost importance for rapid diagnosis. Most forms of RPGN are treated with immunosuppressive regimens which generally consist of high dose steroids in combination with the alkylating agent cyclophosphamide. Some forms also require the use of plasma exchange therapy. Rapid diagnosis and early therapy improves both renal and overall outcome in the affected patients.