• Rare disease characterized by necrotizing granulomas and vasculitis of upper and lower respiratory tracts; also systemic vasculitis with focal necrotizing glomerulonephritis.
  • Triad of upper respiratory tract, lung and kidney involvement is the classical mode of presentation.
  • Systemic vasculitis may be manifested by skin, eye and joint findings.
  • Limited form with long survival when the disease is predominantly restricted to lungs.
  • Etiology is unknown.
Clinical Picture Diagnosis
  • Waxing and waning lung lesions, single or multiple often with cavitation.
  • CXR: Bilateral nodules ranging from 1 to 9 cm in size; may also see diffuse interstitial disease and alveolar hemorrhage.
  • Antineutrophilic cytoplasmic antibodies (ANCA) Percutaneous renal biopsy; lung biopsy.
  • Depending on the organ involved, necrotizing nasal lesions, glomerulonephritis, CNS or skin involvement can be seen on biopsy.
  • Cytotoxic therapy (Cyclophosphamide) with steroids produce rapid reversal of disease.

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