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Double-Positive Anti-GBM/ANCA — ESENeph MCQ

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HardRenal VasculitisDouble-Positive Anti-GBM/ANCAESENeph

A 60-year-old man presents with rapidly progressive renal failure. He has both PR3-ANCA and anti-GBM antibodies positive (double-positive). Kidney biopsy shows crescentic glomerulonephritis with linear IgG staining on IF. What treatment is recommended?

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Correct answer: A — Plasma exchange, cyclophosphamide and glucocorticoids

Double-positive disease (ANCA + anti-GBM) occurs in approximately 5-10% of anti-GBM cases. KDIGO 2024 AAV guideline practice point 9.3.1.10 states: add plasma exchange for patients with overlap syndrome of ANCA-associated vasculitis and anti-GBM disease. The treatment should follow the anti-GBM protocol (plasma exchange + cyclophosphamide + glucocorticoids) given the severity and the presence of anti-GBM antibodies, which mediate the most acute injury.

Reference: https://guidelines.ukkidney.org