- Biopsies of mucosal lesions, skin lesions, lung, or kidney typically are needed to confirm a diagnosis of Wegner granulomatosis, but tissue diagnosis is often difficult unless an open lung biopsy is done.
- A positive c-ANCA (or anti-PR3 antibody) test in the setting of a patient with 2 of 3 classic areas of involvement (i.e., nephritis, pulmonary lesions, sinusitis/otitis) may be sufficient for a clinical diagnosis.
- Aggressive therapy with potent immunosuppressive/cytotoxic therapy is needed for most patients with Wegener granulomatosis. Patients with severe renal or pulmonary manifestations usually require cyclophosphamide for initial disease control.
- After initial remission, most patients should be continued on methotrexate, azathioprine, or mycophenolate as maintenance therapy to reduce the risk of later exacerbation.
- Rituximab appears to be useful for patients with relapsing disease and may eventually be an appropriate initial therapy for many patients.
- Mortality rates are falling as a result of more effective intervention but remain elevated substantially in severe disease.
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