[Wegener's granulomatosis--disease course and treatment response of 18 patients]

Eighteen patients (12 female) with Wegener's granulomatosis (WG) treated from 1982-2001 were studied. Mean duration of patients' symptoms prior to diagnosis was 13.5 months, and mean age 41.2. Lung biopsy confirmed WG in 5 patients, nasal biopsy was made in 12, endobronchial in 3. In 11 of 14 patients cANCAs were found. Prednisone and 2 mg/kg/day doses of oral cyclophosphamide were used to treat 17 patients, prednisone was used alone for 1. Patients with severe symptoms were given i.v. 'pulse' methylprednisolone. Mean duration of treatment was 34.8 months and of follow-up, 71.9 months. A total of 4 patients died: 3 from complete renal failure. Four patients still undergo treatment, improving markedly. Complete remission was achieved in 6 patients. A total of 3 patients relapsed and are recently treated. In another one chronic renal failure developed. Three of them had moderate symptoms at diagnosis but were treated inadequately.

manifestation of WG and severity of symptoms may be different among patients. ANCA is not universally present in all patients with WG. The absence of ANCA does not exclude WG diagnosis. Complete remission can be achieved in WG patients, including the severely ill, if adequate therapy is given. Moderate symptoms at presentation do not justify less intensive treatment.