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

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.

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.