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Wegener’s granulomatosis

Contents of this page:

Illustrations

Adenocarcinoma - chest X-ray
Adenocarcinoma - chest X-ray
Wegener's granulomatosis on the leg
Wegener's granulomatosis on the leg
Respiratory system
Respiratory system

Alternative Names    Return to top

Midline granulomatosis

Definition    Return to top

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

Causes    Return to top

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    Return to top

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:

Exams and Tests    Return to top

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:

Other tests that may be done include:

Treatment    Return to top

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:

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

Other medicines may be prescribed, including:

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.

Outlook (Prognosis)    Return to top

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    Return to top

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.

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

Other complications may include:

When to Contact a Medical Professional    Return to top

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

Prevention    Return to top

There is no known prevention.

References    Return to top

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.

Update Date: 1/13/2009

Updated by: Andrew Schriber, MD, FCCP. Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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