The triad of acute necrotizing granulomas in the upper and lower respiratory tract, necrotizing/granulomatous vasculitis of all small vessels, and glomerulonephritis describes which disease?

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

Answer: (A)

Granulomatosis with polyangiitis (previously Wegener granulomatosis) classically shows a triad of necrotizing granulomatous inflammation in the respiratory tract (both upper and lower airways), necrotizing/granulomatous vasculitis of small vessels, and glomerulonephritis. This combination is highly characteristic because it links airway granulomas with vasculitis seen in the kidneys. Clinically this disease is often associated with c-ANCA (PR3-ANCA) positivity, supporting the diagnosis.

Churg-Strauss (eosinophilic granulomatosis with polyangiitis) would typically have asthma and marked eosinophilia with eosinophil-rich granulomatous inflammation. Microscopic polyangiitis involves small-vessel vasculitis and glomerulonephritis but lacks the granulomatous involvement of the respiratory tract. Kawasaki disease is a medium-vessel vasculitis affecting children, with mucocutaneous findings and coronary artery involvement rather than the described small-vessel granulomatous pattern.