SAT0239 PREDICTION OF LONG-TERM EVOLUTIONARY PROFILES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG–STRAUSS) BASED ON BASELINE AND FOLLOW-UP CHARACTERISTICS

Background Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) control the disease, but GC-dependence is frequent. Evolving concepts distinguish vasculitis-related symptoms from asthma and/or ENT manifestations. That distinction has become even more important since the development of B-cell and eosinophil-targeted therapies. Objectives This study aimed to describe and identify characteristics predicting long-term EGPA outcomes. Methods We set up a multicenter European cohort that included 636 EGPA patients. Based on recent consensus, we distinguished 4 EGPA-evolutionary profiles: GC-dependent asthma and/or ENT manifestations (requiring prednisone >7.5 mg/d), ≥1 vasculitis relapse(s) (excluding asthma and/or ENT flares), both phenotypes, and complete remission (no GC-dependent asthma/ENT signs, no vasculitis relapse and prednisone Results After median follow-up of 63 (IQR 30-110) months, 35.8% had GC-dependent asthma and/or ENT manifestations, 12.9% had ≥1 vasculitis relapse(s), 14.3% had both phenotypes, 14.6% were in complete remission, 14.4% were in partial remission and 7.8% had not reach remission. Patients with GC-dependent asthma/ENT manifestations were younger at diagnosis (p Patients with vasculitis relapse(s) had more frequently neurological manifestations at diagnosis (p=0.002) and MPO-ANCA positivity (p Finally, patients in complete remission were older (p Conclusion EGPA seems to evolve toward distinct phenotypic profiles, which could be identified using baseline and follow-up characteristics. Early identification of those profiles could allow guided choices of the best therapeutic option. Disclosure of Interests Matthias Papo: None declared, Renato A. Sinico: None declared, Vitor Teixeira: None declared, Maria-Letizia Urban: None declared, Juliane Mahrhold: None declared, Sara Monti: None declared, Giulia Cassone: None declared, Franco Schiavon: None declared, Benjamin Seeliger: None declared, Thomas Neumann: None declared, Claus Kroegel: None declared, Matthieu Groh: None declared, Chiara Marvisi: None declared, Maxime Samson: None declared, Thomas Barba: None declared, David Jayne Grant/research support from: David Jayne has received research grants from Chemocentryx, GSK, Roche/Genentech and Sanofi-Genzyme. He has received consultancy fees from Astra-Zeneca, Boehringer-Ingelheim, Chemocentryx, Chugai, GSK, Infla-RX, Insmed and Takeda, Bernhard Hellmich Consultant for: Roche, Speakers bureau: Abbvie, MSD, Roche, Novartis, Pfizer, Carlomaurizio Montecucco Speakers bureau: AbbVie, Bristol-Myers Squibb, Celgene, Sanofi, Genzyme, Lilly, MSD, Pfizer, UCB, Carlo Salvarani Grant/research support from: Roche, Consultant for: Eli Lilly and Company, Roche, Abbvie, Jean-Emmanuel Kahn: None declared, Bernard Bonnotte: None declared, Cecile-Audrey Durel: None declared, Luc Mouthon: None declared, Xavier Puechal: None declared, Loic Guillevin: None declared, Giacomo Emmi: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared