Wegener granulomatosis is estimated to occur in 0.4 per 100,000 individuals. It can occur at any age; its peak incidence is in individuals in their 40s and 50s.
Symptoms
Symptoms in a person with Wegener granulomatosis will reflect where the granulomas have formed:
- Respiratory problems such as chronic sinusitis, ulcers inside the nose, nasal congestion, runny nose, cough, or shortness of breath
- Kidney problems, which show up as protein or blood in the urine
- Fever, weight loss, loss of appetite
- Eye problems such as conjunctivitis and uveitis
- Nervous system problems (34% of individuals) may include numbness, weakness, and tingling in the hands and feet, headaches, seizures, and mental changes.
Diagnosis
If the symptoms suggest Wegener granulomatosis, a test for c-antineutrophil cytoplasmic antibody (ANCA) level can be done. An elevated ANCA level, along with classic symptoms, is 90% sensitive for the diagnosis of Wegener granulomatosis. The diagnosis can be confirmed by taking a tissue sample (biopsy) and examining it for granulomas and tissue damage caused by the disorder.
Treatment
Most individuals with Wegener granulomatosis receive steroids such as prednisone (Deltasone) and cyclophosphamide (Cytoxan) together. Azathioprine (Imuran) or methotrexate may also be given. These drugs all have serious side effects, so usually they are given for short periods of time or only for a few days each month (known as pulse therapy).
For 70% of individuals with Wegener granulomatosis, the medications stop the disorder from becoming worse. However, return of the disorder (relapse) is common. Individuals require close follow-up medical care to monitor their responses to therapy, potential relapses, and side effects of the medications.
Information for this article was taken from:
Scott, T. (2002). Wegeners granulomatosis. eMedicine, accessed at
http://www.emedicine.com/neuro/topic396.htm
