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.

Alternative Names

Midline granulomatosis

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 to 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. The kidney disease can progress rapidly, with kidney failure occurring within months of the initial diagnosis. If untreated, kidney failure and death occur in more than 90% of all patients with Wegener's granulomatosis.

Signs and tests

Treatment

Treatment with corticosteroids, cyclophosphamide, methotrexate, or azathioprine produces long-term remission in over 90% of affected people.

Support groups

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.

Complications

Calling your health care provider

Call your health care provider if chest pain , coughing up blood , blood in the urine , or other symptoms of this disorder are present.

Prevention

No preventive measures are known.