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Alternative namesMidline granulomatosis
DefinitionWegener's Granulomatosis is a rare disorder which causes inflammation of blood vessels ( vasculitis ) in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys. Many other areas of the body may also be affected, with arthritis (joint inflammation) occurring in almost half of all cases. The eyes and skin may also be affected.
Causes, incidence, and risk factors
The cause is unknown, but Wegener's Granulomatosis is thought to be an autoimmune disorder and is often classified as one of the rheumatic diseases. Destructive lesions develop in the upper and lower respiratory tract and the kidney. In the kidney, these lesions cause glomerulonephritis that may result in hematuria (blood in the urine) and kidney failure .
It occurs most often between the ages of 30 and 50, and men are affected twice as often as women. It is rare in children, but has been seen in infants as young as 3 months old.
Early symptoms may include fatigue , malaise (an ill feeling), fever , and a sense of discomfort around the nose and sinuses. Upper respiratory infections such as sinusitis or ear infections frequently precede the diagnosis of Wegener's Granulomatosis. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.
Persistent fever without an obvious cause (fever of undetermined origin -- FUO) may be an initial symptom. Night sweats may accompany the fever. Loss of appetite and weight loss are common. Skin lesions are common, but there is no one characteristic lesion associated with the disease.
Kidney disease is necessary to make the definitive diagnosis of Wegener's Granulomatosis. The urine may be bloody, which often first appears as red or smoky urine. There may be no symptoms, but is easily diagnosed with laboratory studies.
Eye problems develop in a significant number of patients and may range from a mild conjunctivitis to severe inflammation of the eyeball and the tissues around the eyeball. Additional symptoms include:
Signs and tests
TreatmentTreatment with corticosteroids, cyclophosphamide, methotrexate, or azathioprine produces long-term remission in over 90% of affected people.
Support groups with others who suffer from similar diseases may help patients and their families learn about their diseases and adjust to the changes associated with the treatment.
Expectations (prognosis)With treatment, most people recover within months, although chronic renal failure may develop. Without treatment, the disease is usually fatal within a few months of diagnosis.
Calling your health care providerCall your health care provider if chest pain, coughing up blood, blood in the urine, or other symptoms of this disorder are present.
PreventionNo preventive measures are known.
Update Date: 10/22/2003Allen J. Blaivas, D.O, Division of Pulmonary, Critical Care, and Sleep Medicine, University Hospital, Newark, NJ. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT