TY - JOUR T1 - Wegener's Granulomatosis Strictly and Persistently Localized to One Organ Is Rare: Assessment of 16 Patients from the French Vasculitis Study Group Database JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.100518 SP - jrheum.100518 AU - Christian Pagnoux AU - Muriel Stubbe AU - François Lifermann AU - Olivier Decaux AU - Michel Pavic AU - Alice Bérezné AU - Isabelle Delacroix-Szmania AU - Nadine Méaux-Ruault AU - Boris Bienvenu AU - Jean Cabane AU - Loïc Guillevin Y1 - 2010/12/01 UR - http://www.jrheum.org/content/early/2010/11/25/jrheum.100518.abstract N2 - 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. ER -