Rapidly Progressive Glomerulonephritis (RPGN)
Rapidly Progressive Glomerulonephritis (RPGN) is a severe and aggressive form of glomerular disease characterized by a rapid decline in kidney function over days to weeks. It is a true nephrological emergency that requires urgent diagnosis and prompt treatment to prevent irreversible kidney failure. RPGN is uncommon but carries a high risk of progression to end-stage kidney disease if intervention is delayed.
RPGN results from intense immune-mediated injury to the glomeruli, leading to formation of crescents—collections of inflammatory cells and fibrin within Bowman’s capsule—that rapidly impair filtration. It may occur as part of systemic autoimmune diseases, vasculitis, anti–glomerular basement membrane disease, or severe immune complex–mediated glomerulonephritis. In some patients, RPGN develops without clear systemic involvement.
Patients typically present with rapidly worsening kidney function, reduced urine output, blood and protein in the urine, and rising blood pressure. Systemic symptoms such as fatigue, fever, joint pain, or respiratory complaints may be present depending on the underlying cause. Laboratory evaluation often reveals rapidly rising serum creatinine, hematuria with red blood cell casts, proteinuria, and abnormal immune markers.
Diagnosis requires urgent evaluation with blood and urine tests and kidney biopsy, which is essential to confirm crescentic glomerulonephritis and identify the underlying mechanism. Early biopsy allows targeted therapy and improves the likelihood of renal recovery.
Management of RPGN involves aggressive immunosuppressive therapy to halt inflammatory injury, combined with supportive renal care. Treatment strategies are individualized based on biopsy findings and disease severity. Many patients require close monitoring in a hospital setting. Renal replacement therapy may be necessary in cases of severe kidney failure or metabolic complications.
Prognosis depends on the speed of diagnosis, extent of crescent formation, and response to therapy. Early nephrology involvement is critical, as timely treatment can preserve kidney function and prevent progression to permanent renal failure. Long-term follow-up is required due to risk of relapse and chronic kidney disease.
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