Wegener’s granulomatosis (also called Wegener’s disease) is an inflammatory disorder of small arteries and veins that leads to the development of granulomas, or nodules, within blood vessels and other tissues. Autoimmunity probably plays a role in the development of this process, which affects about 1 in 25,000 people. (The Merck Manual, 18th Edition. Wegener's granulomatosis. 2006:281-283)
Since the 1970s, scientists have been actively investigating the cause of Wegener’s disease and attempting to develop successful treatments that don’t carry a heavy burden of adverse effects. However, the underlying etiology remains elusive, and the mainstay of therapy still consists of powerful immunosuppressant drugs that are poorly tolerated by many patients.
WG can affect any organ in the body, but it most commonly involves the respiratory tract (nose, sinuses, trachea, and lungs) and the kidneys. The disease affects men and women equally, it can occur at any age (although the mean age at onset is 40 years), and it occurs more often in Caucasians than in other ethnic groups.
Signs and Symptoms of Wegener’s Granulomatosis
Wegener’s can begin suddenly, or its onset can be insidious; the disease may take years to develop fully. Manifestations—many of which resemble those of other autoimmune conditions—may include:
- Fever
- Fatigue
- Loss of appetite
- Weight loss
- Migratory arthritis and joint pain
- Nasal discharge, either purulent or bloody
- Headache due to sinus involvement
- Ulcerations of the nasal mucous membranes
- Recurrent bacterial infections of the nose
- Recurrent ear infections
- Chronic cough, sometimes with bloody sputum
- Skin nodules and ulcerations
- Excessive tearing due to obstruction of nasolacrimal ducts
- Conjunctivitis
- Bulging of the eyes due to development of orbital granulomas
- Shortness of breath
- Angina and heart attack due to inflammation of coronary arteries
- Numbness or abnormal sensations due to nerve involvement
- High blood pressure, usually due to kidney involvement
- Anemia
- Renal insufficiency or kidney failure
How is Wegener’s Granulomatosis Diagnosed?
- Blood tests: Erythrocyte sedimentation rate is often elevated; white count is commonly high; anemia may be severe; antineutrophilic cytoplasmic antibodies (ANCA) are often present—specificity for Wegener’s is high with this test, but a positive ANCA screen is not diagnostic.
- When the kidneys are involved, urinalysis may reveal hematuria, proteinuria, and casts of red blood cells.
- X-ray studies of lungs or nasal sinuses may be helpful.
- Biopsy of an involved organ or tissue is the key to confirming Wegener’s disease. Lung biopsy is the most commonly performed procedure.
- Early diagnosis is important, because prompt treatment can induce remission and prevent complications or even death.
Treatment for Wegener’s Granulomatosis
- The first line of therapy for Wegener’s disease usually consists of high-dose prednisone and cyclophosphamide to suppress the immune system. Other drugs, such as the antibiotic Bactrim, can be added to this regimen to reduce the risks of future relapse.
- Because cyclophosphamide is a relatively toxic drug, it is often replaced by other medications—drugs like methotrexate or azathioprine—once remission is attained. These drugs can sometimes be tapered after a couple of years of treatment.
- More recently, successes with immunomodulating drugs like intravenous immunoglobulins or rituximab have offered promise to patients suffering from Wegener’s granulomatosis.
Wegener’s granulomatosis is an uncommon disease with protean manifestations and the potential for life-threatening complications. Treatment is aimed at reducing vascular inflammation by suppressing the immune system with potent medications.
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