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Rapidly progressive glomerulonephritis — ABIM Board MCQ

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HardNephrologyRapidly progressive glomerulonephritisABIM Board

A 58-year-old man is hospitalized with hemoptysis, dyspnea, and oliguria. His serum creatinine has risen from 1.0 to 7.6 mg/dL over 2 weeks, and hemodialysis is initiated for refractory hyperkalemia. Urinalysis shows dysmorphic erythrocytes and erythrocyte casts. Chest CT demonstrates diffuse alveolar hemorrhage. Serum anti–glomerular basement membrane antibodies are positive, and kidney biopsy shows crescentic glomerulonephritis with linear IgG deposition along the glomerular basement membrane. Which of the following is the most appropriate disease-directed treatment?

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Correct answer: D — Plasma exchange, glucocorticoids, and cyclophosphamide

Explanation lettering: D = shown as A · C = shown as B · B = shown as C · A = shown as D

This patient has anti-GBM disease causing rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. Treatment should begin urgently with plasma exchange to remove circulating anti-GBM antibodies, cyclophosphamide to suppress further antibody production, and glucocorticoids to control inflammation. Dialysis dependence and extensive crescent formation predict poor renal recovery but do not justify withholding disease-directed therapy when active pulmonary hemorrhage is present, because treatment can be lifesaving. Omitting plasma exchange (B or D) leaves pathogenic antibodies in circulation; omitting cyclophosphamide (C) permits continued antibody production. Rituximab is generally reserved for selected patients who cannot receive standard cyclophosphamide-based therapy. Hemodialysis and supportive care alone (E) address complications but do not treat the pulmonary capillaritis or underlying autoimmune process.

Reference: National Institute of Diabetes and Digestive and Kidney Diseases. Anti-GBM (Goodpasture’s) Disease, section “How do health care professionals treat anti-GBM disease?” Last reviewed April 2020. https://www.niddk.nih.gov/health-information/kidney-disease/glomerular-disease/anti-gbm-goodpastures-disease