Wegener granulomatosis

• sinuses and upper respiratory tract (100%)
  • mucoperiosteal thickening
  • rhinorrhea and epistaxis
  • may destroy bone + cartilage

• lungs
  • necrotizing granulomata
  • multiple round nodules (2 mm - 9 cm)
  • alveolar infiltrates
  • may cavitate

• kidneys (83%)
  • glomerulonephritis ==> necrosis of capillary tuft

• generalized necrotizing vasculitis of arteries + veins
  • auto-immune: basement membrane
  • almost always involves lungs

• M = F, 30-50 y/o
• sx: cough, hemoptysis, fever, wt loss, multiple resp. infections
• tx: cyclophosphamide, corticosteroids (90% complete remission)
• 90% mortality within 2 years if untreated (renal>pulmonary failure)

© 2002, C.E. Kahn, Jr.
2002-04-18