A 44-year-old patient presents with hematuria, hemoptysis, and head/ENT involvement. Which vasculitis is most likely?

• A. Granulomatosis with polyangiitis (GPA)
• B. Polyarteritis nodosa
• C. Churg-Strauss syndrome
• D. Microscopic polyangiitis

Answer: (A)

The key idea is recognizing the combination of upper airway (ENT) involvement, lung hemorrhage, and kidney involvement. Granulomatosis with polyangiitis is an ANCA-associated vasculitis that classically causes necrotizing granulomatous inflammation in the respiratory tract (leading to chronic sinusitis, nasal crusting, otitis, or even saddle-nose deformity) and necrotizing vasculitis of small to medium vessels in the kidneys, producing hematuria, often from rapidly progressive glomerulonephritis. Lung disease in this condition can manifest as hemoptysis from pulmonary capillaritis or cavitary nodules.

This pattern differs from the others: polyarteritis nodosa typically involves medium-sized arteries and spares the lungs, and lacks the granulomatous upper airway disease; Churg-Strauss (eosinophilic granulomatosis with polyangiitis) presents with asthma, eosinophilia, and allergic features, often with MPO-ANCA; microscopic polyangiitis causes small-vessel vasculitis with GN and pulmonary hemorrhage but generally lacks granulomatous ENT involvement. Because the patient’s mix of hematuria, hemoptysis, and head/ENT involvement aligns most closely with granulomatosis with polyangiitis, that is the best fit.