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Granulomatosis with polyangiitis — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologyGranulomatosis with polyangiitisABIM Board

A 49-year-old man has hemoptysis, dyspnea, sinus crusting, and rapidly worsening kidney function. Urinalysis shows dysmorphic RBCs and RBC casts. CT chest shows bilateral cavitary nodules. PR3-ANCA is positive. Kidney biopsy shows pauci-immune necrotizing crescentic glomerulonephritis. Which of the following is the most appropriate management?

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Correct answer: C — High-dose glucocorticoids plus rituximab or cyclophosphamide

Granulomatosis with polyangiitis causing pulmonary-renal syndrome is organ-threatening ANCA-associated vasculitis. Induction therapy requires high-dose glucocorticoids plus rituximab or cyclophosphamide; plasma exchange is individualized.

Reference: 2021 ACR/Vasculitis Foundation ANCA-Associated Vasculitis Guideline