Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: Evidence-based recommendations

Idiopathic crescentic glomerulonephritis (GN) often presents with a rapid l oss of renal function and pathology showing extensive crescent formation. T he disease is caused by different immunopathogenetic mechanisms, pauci-immu ne, often antineutrophil cytoplasmic antibody (ANCA)-positive microvasculit is, antiglomerular basement membrane (GBM) antibody disease, and immune com plex formation. Historical reviews reveal poor renal prognosis, even after treatment with oral steroids and cytotoxic drugs. Prognosis has improved in the last decade. In this article, evidence-based recommendations for manag ement are presented. Because of the high risk of end-stage renal disease (E SRD), early aggressive therapy is recommended, despite weak supporting evid ence. Treatment for anti-GEM antibody-induced crescentic GN should be initi ated early and should include pulse methylprednisolone, a two-week course o f plasmapheresis and two months of treatment with corticosteroids and cyclo phosphamide (grade B and C). Treatment for pauci-immune crescentic GN shoul d be pulse methylprednisolone, followed by oral corticosteroids and cycloph osphamide for 6 to 12 months (grade B). Recurrences can be managed similarl y (grade B), along with appropriate supportive therapy. In patients who dev elop ESRD, successful transplantation can be performed. Diffuse endocapilla ry proliferative GN is classically postinfectious. It generally has a good prognosis when no crescent formation occurs. Adult patients with persistent proteinuria, hypertension, and renal function impairment need careful foll ow-up and management to modify progressive hemodynamic injury.