Which disease is most commonly associated with nasal/sinus involvement and kidney disease, reflecting granulomatous inflammation of the airways and kidneys?

Which disease is most commonly associated with nasal/sinus involvement and kidney disease, reflecting granulomatous inflammation of the airways and kidneys?

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

Answer: (B)

Granulomatosis with polyangiitis (formerly Wegener granulomatosis) is the vasculitis that most clearly links granulomatous inflammation of the airways with kidney disease. It characteristically causes chronic nasal/sinus symptoms with destructive nasal lesions and rapidly progressive glomerulonephritis. Histology shows necrotizing granulomatous inflammation in the respiratory tract along with necrotizing vasculitis in the kidneys, often with c-ANCA (PR3-ANCA) positivity. This combination of upper airway granulomatous involvement and renal disease is the hallmark. Microscopic polyangiitis lacks granulomatous airway lesions and more commonly presents with pauci-immune glomerulonephritis and pulmonary capillaritis, without the classic nasal sinus destruction. Churg-Strauss (EGPA) involves eosinophilia and asthma with granulomatous vasculitis but has a different clinical pattern and serology. Kawasaki disease is a pediatric vasculitis with mucocutaneous features and coronary involvement, not the granulomatous airway disease paired with kidney pathology described here.

Granulomatosis with polyangiitis (formerly Wegener granulomatosis) is the vasculitis that most clearly links granulomatous inflammation of the airways with kidney disease. It characteristically causes chronic nasal/sinus symptoms with destructive nasal lesions and rapidly progressive glomerulonephritis. Histology shows necrotizing granulomatous inflammation in the respiratory tract along with necrotizing vasculitis in the kidneys, often with c-ANCA (PR3-ANCA) positivity. This combination of upper airway granulomatous involvement and renal disease is the hallmark.

Microscopic polyangiitis lacks granulomatous airway lesions and more commonly presents with pauci-immune glomerulonephritis and pulmonary capillaritis, without the classic nasal sinus destruction. Churg-Strauss (EGPA) involves eosinophilia and asthma with granulomatous vasculitis but has a different clinical pattern and serology. Kawasaki disease is a pediatric vasculitis with mucocutaneous features and coronary involvement, not the granulomatous airway disease paired with kidney pathology described here.