Wegener’s granulomatosis

• a necrotizing vasculitis

• triad

  • necrotizing vasculitis of small- to medium-sized vessels, particularly in the lung

  • acute necrotizing granulomas of the upper respiratory tract

  • necrotizing glomerulonephritis

• ding-dong: associated with c-ANCA, which is a sensitive and specific marker

• males > females, 40-50 years old

Clinical features

• pneumonitis

• ulcers of the nasopharynx

• renal disease

• lots of different stuff

Histology

• necrotizing vasculitis: vessel has been completely occluded and there is fibrinous necrosis of vessel wall

• granulomatous inflammation—eosinophils, neutrophils

Clinical course

• malignant disease: 80% of patients die within one year with no treatment

• rx with steroids and immunosuppressants, inc. cyclophosphamide

Category: Medical Subject Notes , Pathology Notes