Granulomatosis with Polyangiitis (GPA; Wegener's Granulomatosis) - Diagnosis and Management Summary

ANCA-Associated (Pauci-Immune) Small-Vessel Vasculitis - Necrotizing granulomatous inflammation of arteries, capillaries, and veins, usually involving upper and lower airways (90%) and kidney (80%), +/- cutaneous (leukocytoclastic vasculitis)

 • Dx: sinus CT (+/- bone erosions), Bx w/ granulomatous inflammation of vessel walls, 90% + c-ANCA

 • Rx: limited disease: MTX + prednisone; severe disease: IV pulse steroids x3 days (with oral taper) + RTX or CYC (+/- plasma exchange)



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