Objective: To study the frequency and characteristics of patients with Wegener's granulomatosis (WG) strictly and persistently localized to one organ.
Methods: Retrospective analysis of the French Vasculitis Study Group (FVSG) WG cohort.
Results: 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.
Conclusion: Strictly and persistently localized WG is uncommon. Optimal treatment remains to be determined.