• 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.

Therapy

• Cytotoxic therapy (Cyclophosphamide) with steroids produce rapid reversal of disease.