Wegener's granulomatosis Health Article

Definition

Wegener's granulomatosis is a rare disorder in which certain blood vessels become swollen and inflamed. The inflammation makes it hard for blood to flow. The disease mainly affects the blood vessels in the nose, sinuses, ears, lungs, and kidneys, although other areas may be involved.

Alternative Names

Midline granulomatosis

Causes, incidence, and risk factors

The cause is unknown. Wegener's granulomatosis is thought to be an autoimmune disorder. Arthritis occurs in about half of all those with this disease.

Wegener's granulomatosis is most common in middle-aged adults. It is rare in children, but has been seen in infants as young as 3 months old.

Symptoms

Frequent sinusitis is the most common symptom. Other early symptoms include a fever that continues 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 many patients. The eye problems range from mild conjunctivitis to severe swelling of the eye.

Other symptoms include:

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

Signs and tests

Your doctor may order a blood test that looks for a disease-fighting proteins called antineutrophil cytoplasmic antibodies (ANCA), which are often found in persons with active Wegener's granulomatosis. However, it is not foolproof -- the test may be negative in some patients.

Urinalysis is done to look for signs of kidney disease such as protein and blood in the urine. The presence of kidney disease is necessary to definitely diagnose Wegener's granulomatosis.

A biopsy is the only way to definitely diagnose this disease. The exact type of biopsy depends on which area of the body the doctor wants to look at. Your doctor may suggest one of the following:

• Open lung biopsy
• Upper airway biopsy
• Nasal mucosal biopsy
• Bronchoscopy with transtracheal biopsy

Other tests that may be done include:

• Chest x-ray
• Chest CT scan
• Bone marrow aspiration