Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database.

Abstract

To study the frequency and characteristics of patients with Wegener's granulomatosis (WG) strictly and persistently localized to one organ. Retrospective analysis of the French Vasculitis Study Group (FVSG) WG cohort. Sixteen patients (3.2% of the cohort) were identified who had isolated lung nodules, ear-nose-throat, or ocular involvement that did not progress to systemic disease (median followup, 58 mo) over the period of observation. Ten received first-line therapy with cyclophosphamide, which was effective in 4. Cotrimoxazole alone achieved remission in one, combined with corticosteroids in 3. Eight required subsequent treatments because of first-line failure or relapse. Strictly and persistently localized WG is uncommon. Optimal treatment remains to be determined.