Rapidly Progressive Glomerulonephritis

Abstract

Rapidly progressive glomerulonephritis (RPGN) is a syndrome characterized by a swift decline in kidney function, often over a few months, accompanied by features of nephritic syndrome. It can result in decreased urine output and commonly involves the presence of extensive crescents in kidney biopsies. RPGN is classified into 3 main types based on immune deposit distribution and visualization through immunofluorescence and electron microscopy: antiglomerular basement membrane disease, immune complex glomerulonephritis, and pauci-immune glomerulonephritis. Early diagnosis and prompt treatment are critical to prevent progression to ESRD. Standard treatment options for RPGN include glucocorticoids, cyclophosphamide, or rituximab, with plasma exchange especially important for antiglomerular basement membrane disease and select cases of ANCA-associated vasculitis. Clinical trials for glomerular diseases have primarily excluded patients with RPGN or dialysis dependence. Establishment of clinical registries is required for the optimization of therapeutic protocols for the treatment of RPGN.