Which disease is defined by necrotizing granulomatous inflammation of the respiratory tract with glomerulonephritis?

• A. Wegener granulomatosis
• B. Microscopic polyangiitis
• C. Churg-Strauss Syndrome
• D. Kawasaki disease

Answer: (A)

Granulomatosis with polyangiitis (formerly Wegener granulomatosis) is defined by necrotizing granulomatous inflammation of the respiratory tract together with glomerulonephritis. This means there are granulomatous lesions in the nasal/sinus and lung regions accompanied by a necrotizing vasculitis that can involve the kidneys, leading to kidney damage. Clinically you’d expect upper airway symptoms (nasal crusting, sinusitis, possibly saddle-nose deformity), lung involvement, and rapidly progressive glomerulonephritis. Many patients have c-ANCA (PR3-ANCA) antibodies, which supports this diagnosis. Other vasculitides may affect the lungs and kidneys but don’t typically present with this exact combination of respiratory tract granulomatous inflammation plus glomerulonephritis. Kawasaki disease is a pediatric vasculitis with mucocutaneous findings and coronary artery aneurysms, not the described triad.