5-Minute Clinical Consult : Wegener Granulomatosis

Wegener Granulomatosis

Clinical Pearls

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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