Wegener granulomatosis


We·ge·ner gran·u·lo·ma·to·sis

(veg'ĕn-ĕr), a disease occurring mainly in the fourth and fifth decades that is characterized by necrotizing granulomas, vasculitis, and ulceration of the upper respiratory tract, with purulent rhinorrhea and nasal obstruction, and sometimes with otorrhea, hemoptysis, pulmonary infiltration and cavitation, and fever. Exophthalmos, involvement of the larynx and pharynx, and glomerulonephritis may occur. The underlying condition, a vasculitis affecting small vessels, is possibly caused by an immune disorder. Cases in which only the lungs are involved have been seen; isolated tracheal stenosis is increasingly recognized.
See also: lymphomatoid granulomatosis.

We·ge·ner gran·u·lo·ma·to·sis

(vā'ge-ner gran'yū-lō'mă-tō'sis) A disease, occurring mainly in the fourth and fifth decades, characterized by necrotizing granulomas and ulceration of the upper respiratory tract, with purulent rhinorrhea, nasal obstruction, and sometimes with otorrhea, hemoptysis, pulmonary infiltration and cavitation, and fever; exophthalmos, involvement of the larynx and pharynx, and glomerulonephritis may occur; the underlying condition is a vasculitis affecting small vessels, and is possibly due to an immune disorder.

Wegener granulomatosis

, Wegener syndrome (veg'en-er) [Frederich Wegener, Ger. pathologist, 1843–1917] A systemic necrotizing vasculitis marked by pneumonitis and glomerulonephritis; small and medium-sized blood vessels throughout the body may be affected. The average age of onset is 40, and the disease affects men more often than women.

Etiology

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.

Symptoms

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.

Treatment

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.

 See: granuloma

Wegener,

Friedrich, German pathologist, 1907-1990. Wegener granulomatosis - characterized by necrotizing granulomas and ulceration of the upper respiratory tract, with purulent rhinorrhea, nasal obstruction, and sometimes with otorrhea, hemoptysis, pulmonary infiltration and cavitation, and fever.