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Anti-GBM Disease Treatment — SCE Rheumatology MCQ

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HardRheumatology EmergenciesAnti-GBM Disease TreatmentSCE Rheumatology

A 60-year-old woman with known anti-GBM disease presents with rapidly progressive glomerulonephritis and pulmonary haemorrhage. Anti-GBM antibodies are strongly positive. ANCA is negative. What is the first-line treatment for anti-GBM disease?

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Correct answer: C — Plasma exchange, cyclophosphamide and glucocorticoids

Anti-GBM disease (Goodpasture disease when involving both lungs and kidneys) requires urgent combined treatment: plasma exchange (daily or alternate day for 2-3 weeks to rapidly remove circulating anti-GBM antibodies) plus Cyclophosphamide and glucocorticoids (to suppress new antibody production). KDIGO 2021/2024 guidelines recommend this triple therapy approach. Treatment must be started immediately as renal prognosis depends on residual renal function at presentation. Patients presenting with creatinine >500 micromol/L requiring dialysis rarely recover renal function. The disease is usually monophasic with low relapse rates once antibodies are cleared. Anti-GBM/ANCA overlap occurs in 10-30% requiring consideration of maintenance immunosuppression.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/musculoskeletal-conditions