Alternate Names: Midline granulomatosis

Causes and Risks: The cause is unknown, but it is thought to be due to an autoimmune disorder and is often classified as one of the rheumatic diseases. Wegener’s is characterized by destructive lesions that develop in the upper and lower respiratory tract and the kidney. In the kidney these lesions cause a glomerulonephritis that may result in kidney failure. It occurs most often between the ages of 30 to 50. It is rare in children, but has been seen in infants as young as 3 months old.

Early symptoms are often systemic and may include fatigue, malaise (an ill feeling), fever, and a sense of discomfort around the nose and sinuses. Persistent fever without obvious cause (fever of undetermined origin - FUO) may be an initial finding. Night sweats may accompany the fever. Anorexia and weight loss are common. Significant symptoms often appear as pulmonary manifestations: cough, bloody sputum, and progressive breathing difficulties (later requiring oxygen). Involvement of the sinuses and nose may produce a bloody discharge, pain, and ulcers and sores around the opening of the nose. Skin lesions are common, but there is no one characteristic lesion associated with the disease. Kidney disease may have no symptoms but is readily 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.

Prevention: No preventive measures are known.

Symptoms:

• fever
• bloody discharge from the nose
• pain over the sinuses
• lesions (ulcers, sores, and crusting) in and around opening of the nose
• cough
• bloody sputum
• shortness of breath
• chest pain
• blood in the urine
• conjunctivitis or other eye problems
• rashes
• loss of appetite
• weight loss
• weakness
• fatigue
• joint pains
• wheezing
• coughing up blood

Signs and Tests:

• biopsy of abnormal tissue, which may include
  • open lung biopsy
  • upper airway biopsy
  • nasal mucosal biopsy
  • bronchoscopy with transtracheal biopsy
• urinalysis (protein and blood in the urine [proteinuria and hematuria])
• chest X-ray (cavity formation, pulmonary infiltrates)
• bone marrow aspiration (may be done)

Treatment: Treatment with corticosteroids, cyclophosphamide, and azathioprine produces long-term remission in approximately 90% of affected people.

Prognosis: With treatment, most people recover within a year, although chronic renal failure may develop. Without treatment, the disease is fatal within a few months of diagnosis.

Complications:

• chronic renal failure
• inflammation of the eyes
• nasal septum perforation (hole)
• rash
• side effects of medications used to treat the disease

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