Granulomatosis with polyangiitis (GPA) most characteristically involves which combination of findings, and what is the associated ANCA pattern?

• A Asthma + eosinophilia + neuropathy; c-ANCA (PR3-ANCA)
• B Pauci-immune glomerulonephritis alone; p-ANCA (MPO-ANCA)
• C Retinal vasculitis + uveitis + renal disease; ANA positive
• D Upper respiratory + lower respiratory + glomerulonephritis; c-ANCA (PR3-ANCA) ✓

Explanation

GPA (formerly Wegener's granulomatosis) classically involves the triad: upper respiratory tract (saddle-nose deformity, nasal septum perforation, sinusitis), lower respiratory tract (cavitating lung nodules), and kidneys (pauci-immune crescentic GN). The ANCA pattern is c-ANCA, directed against proteinase-3 (PR3). Asthma + eosinophilia + neuropathy describes eosinophilic GPA (Churg-Strauss), which is MPO-ANCA (p-ANCA) positive. Microscopic polyangiitis is also p-ANCA/MPO but lacks granulomas and upper airway involvement.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.