OBSOLETE: Rapidly progressive glomerulonephritis

Rapidly progressive glomerulonephritis (RPGN), also known as crescentic glomerulonephritis, is a severe kidney condition characterized by a rapid decline in kidney function over days to weeks. It affects the glomeruli — the tiny filtering units within the kidneys — leading to crescent-shaped formations of cells visible on kidney biopsy. Key symptoms include blood in the urine (hematuria), protein in the urine (proteinuria), reduced urine output, swelling (edema), high blood pressure, and rapidly worsening kidney failure. Patients may also experience fatigue, nausea, and general malaise as kidney function deteriorates. RPGN is not a single disease but rather a clinical syndrome that can result from several underlying causes, broadly classified into three types: anti-glomerular basement membrane (anti-GBM) disease (Type I), immune complex-mediated disease (Type II, associated with conditions such as lupus nephritis or IgA nephropathy), and pauci-immune glomerulonephritis (Type III, often associated with ANCA-associated vasculitis). Because of this heterogeneity, RPGN is considered an obsolete grouping in Orphanet (code 280569), with patients now classified under more specific diagnostic entities. Treatment depends on the underlying cause but typically involves aggressive immunosuppressive therapy, including high-dose corticosteroids, cyclophosphamide, and in some cases plasma exchange (plasmapheresis), particularly in anti-GBM disease. Rituximab has emerged as an alternative in ANCA-associated forms. Early diagnosis and prompt treatment are critical, as delayed intervention can lead to irreversible kidney damage requiring dialysis or kidney transplantation. Prognosis varies significantly depending on the underlying etiology, the degree of kidney damage at presentation, and the timeliness of treatment initiation.

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