Wegener’s granulomatosis

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

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

Exams 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 sometimes 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

Treatment

Your doctor may suggest treatment with glucocorticoids (such as prednisone) which are given along with other medicines that slow down the immune response.

Medicines used to treat Wegener's granulomatosis include:

• Azathioprine (Imuran)
• Cyclophosphamide (Cytoxan)
• Methotrexate

These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor.

Other medicines may be prescribed, including:

• Bisphosphonate (Fosamax) to prevent bone loss caused by prednisone
• Folic acid or folinic acid (leucovorin) -- if you are taking methotrexate
• Trimethoprim/sulfamethoxazole to prevent lung infections

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.

Outlook (Prognosis)

Without treatment, people with this disease can die within a few months.

With treatment, the outlook for most patients is good. Studies have shown that most people who receive corticosteroids and cyclophosphamide get much better.

However, the disease may return in about half of all patients. In these cases, the disease usually comes back within 2 years of stopping treatment.

Possible Complications

Complications usually result from lack of treatment. Patients with Wegener's granulomatosis 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. Kidney function may not improve even when the condition is controlled by medicines.

If untreated, kidney failure and death occur in more than 90% of patients.

Other complications may include:

• Eye swelling
• Lung failure
• Nasal septum perforation (hole inside the nose)
• Side effects from medicines used to treat the disease

When to Contact a Medical Professional

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

Prevention

There is no known prevention.

References

Channick RN, Rubin LJ. Pulmonary Vaculitis and Primary Pulmonary Hypertension. In: Mason RJ, Murray JF, Broaddus VC, Nadel JA, eds. Textbook of Respiratory Medicine. 4th ed. Philadelphia, Pa: Saunders Elsevier; 2005: 1462-1463.

Calabrese LH, Molloy ES, Duna G. Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. In: Firestein GS, Budd RC, Harris ED Jr., McInnes IB, Ruddy S, eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008: Chap 82.

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