Wegener's granulomatosis Health Article

Definition

Wegener's granulomatosis is a rare disorder in which blood vessels become inflamed, making it hard for blood to flow.

Alternative Names

Midline granulomatosis

Causes, incidence, and risk factors

Wegener's granulomatosis mainly affects blood vessels in the nose, sinuses, ears, lungs, and kidneys, although other areas may be involved.

The cause is unknown. It is thought to be an autoimmune disorder.

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 a general ill feeling ( malaise).

Chronic ear infections are common. 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 changes are also common, but there is no one typical lesion associated with the disease.

There may be symptoms of kidney disease. The urine may be bloody.

Eye problems develop in many people with Wegener's granulomatosis. The eye problems range from mild conjunctivitis to severe swelling of the eye.

Other symptoms include:

• Chest pain
• Cough, with or without blood
• Joint pain
• Shortness of breath
• Weakness
• Wheezing

Signs and tests

Your doctor may order a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which are often found in people 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. Sometimes urine is collected over 24 hours to better check how the kidneys are working.

A biopsy is needed to confirm the diagnosis. 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:

• Bronchoscopy with biopsy
• Kidney biopsy
• Nasal mucosal biopsy
• Open lung biopsy
• Skin biopsy
• Upper airway biopsy

Other tests that may be done include:

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