Wegener’s granulomatosis

• Illustrations
• Alternative names
• Definition
• Causes, incidence, and risk factors
• Symptoms
• Signs and tests
• Treatment

• Support Groups
• Expectations (prognosis)
• Complications
• Calling your health care provider
• Prevention
• References

Alternative names    Return to top

Definition    Return to top

Wegener's granulomatosis is a rare disorder that causes blood vessels in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys to become swollen and inflammed. The eyes, skin, and joints may also be affected. Arthritis occurs in about half of all cases.

Causes, incidence, and risk factors    Return to top

The cause is unknown. Wegener's granulomatosis is thought to be an autoimmune disorder.

Patients with this disease develop sores (lesions) in the respiratory tract and the kidneys. Kidney lesions cause glomerulonephritis, which may result in blood in the urine and kidney failure.

Kidney disease can quickly get worse. If untreated, kidney failure and death occur in more than 90% of patients.

Wegener's granulomatosis is most common in middle-aged adults. 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.

Symptoms    Return to top

Frequent sinusitis is the most common symptom. Other early symptoms include persistent fever without an obvious cause, night sweats, fatigue, and  malaise (an ill feeling).

Chronic ear infections may also come before the diagnosis of Wegener's granulomatosis. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose.

Loss of appetite and weight loss are common. Skin lesions typically occur, but there is no one typical lesion associated with the disease.

Symptoms of kidney disease may be present (but not always). The urine may be bloody, and often first appears as red or smoky urine.

Eye problems develop in a significant number of patients. They may range from mild conjunctivitis to severe swelling of the eye.

Other symptoms include:

• Weakness
• Cough, or coughing up blood
• Bloody sputum
• Shortness of breath
• Wheezing
• Chest pain
• Rashes
• Joint pains

Signs and tests    Return to top

• Biopsy of abnormal tissue:
  • Open lung biopsy
  • Upper airway biopsy
  • Nasal mucosal biopsy
  • Bronchoscopy with transtracheal biopsy
  • Kidney biopsy
• Urinalysis to look for signs of kidney disease such as protein and blood in the urine.  The presence of kidney disease is necessary to make a definitive diagnosis of Wegener's granulomatosis.
• Chest x-ray  
• Bone marrow aspiration
• Blood tests to look for antibodies that the body makes against its own tissue.

Treatment    Return to top

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

Support Groups    Return to top

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)    Return to top

With treatment, most people recover within months. However, some may develop chronic renal failure.

Without treatment, patients can die within a few months.  

Complications    Return to top

• Chronic kidney failure
• Hemoptysis (coughing up blood)
• Respiratory failure
• Inflammation of the eyes
• Nasal septum perforation (hole inside the nose)
• Rash

Medications used to treat the disease can cause side effects, which may also lead to complications.

Calling your health care provider    Return to top

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

Prevention    Return to top

No preventive measures are known.

References    Return to top

Murray J, Nadel J. Textbook of Respiratory Medicine. 3rd ed. Philadelphia, Pa: W.B. Saunders Company; 2000: 1462-1463.