WEGENERS GRANULOMATOSIS
- 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.