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See also: lymphomatoid granulomatosis.
We·ge·ner gran·u·lo·ma·to·sis(vā'ge-ner gran'yū-lō'mă-tō'sis)
Wegener granulomatosis, Wegener syndrome (veg'en-er)
The precise etiology is unknown. Autoantibodies have been identified in the blood of approx. 90% of patients. Granulomas may be present in the lung, upper respiratory tract, and small arteries and veins. Localized or diffuse inflammatory patches are seen in the glomerular capillaries of the kidney.
Chronic pneumonitis and glomerulonephritis are the most prominent signs; ulcerations of the nasopharyngeal mucosa also are common. Other signs and symptoms include muscle and joint pain, skin rashes, fever, and neuropathy.
Suppressive immunotherapeutic drugs such as cyclophosphamide and corticosteroids are used to control the disease. Trimethoprim-sulfamethoxazole may prevent relapses. There is a 1-year, 80% mortality rate in untreated patients; when treatment is effective, patients can live normal lifespans. Those with diffuse glomerular damage may develop chronic renal failure.