Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry
Michele Iudici, Christian Pagnoux, Delphine S. Courvoisier, Pascal Cohen, A. Néel, Achille Aouba, François Lifermann, M. Ruivard, O. Aumaître, Bernard Bonnotte, F. Maurier, Thomas Le Gallou, É. Hachulla, Alexandre Karras, C. Khouatra, Noémie Jourde‐Chiche, Jean‐François Viallard, Claire Blanchard‐Delaunay, Pascal Godmer, Alain Le Quellec, T. Quéméneur, Claire de Moreuil, Alexis Régent, Benjamin Terrier, Luc Mouthon, Loı̈c Guillevin, Xavier Puéchal, the French Vasculitis Study Group
Abstract
OBJECTIVE: To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA). METHODS: EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients' characteristics at diagnosis and long-term outcomes were analysed and compared. RESULTS: Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA-positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients' estimated relapse-free-survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P<0.02). Over a median follow-up of 3.5 years, 18 (22.8%) L-GPA progressed to S-GPA, either as a relapse after a period in remission or more frequently in the context of refractory disease. L-GPA patients experienced more ENT-related damage. CONCLUSIONS: The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement.