SAT0239 PREDICTION OF LONG-TERM EVOLUTIONARY PROFILES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG–STRAUSS) BASED ON BASELINE AND FOLLOW-UP CHARACTERISTICS
Authors: Matthias Papo, Renato A. Sinico, Vítor Teixeira, Maria-Letizia Urban, Juliane Mahrhold, Sara Monti, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Bernhard Hellmich, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cécile-Audrey Durel, Luc Mouthon, Xavier Puéchal, Loïc Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> 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. <h3>Objectives</h3> This study aimed to describe and identify characteristics predicting long-term EGPA outcomes. <h3>Methods</h3> 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 &gt;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 &lt;5 mg/d at last follow-up). Baseline and follow-up characteristics predicting those outcomes were analyzed. <h3>Results</h3> 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 (<i>p</i>&lt;0.0001), had more frequent GC-treated asthma before overt EGPA (<i>p</i>=0.002), had more ENT manifestations (<i>p</i>=0.01) and less frequent MPO-ANCA (<i>p</i>&lt;0.0001). Their daily GC dose was higher at every time point (<i>p</i>&lt;0.0001), and they had more frequent active asthma at last follow-up (<i>p</i>&lt;0.0001). Patients with vasculitis relapse(s) had more frequently neurological manifestations at diagnosis (<i>p</i>=0.002) and MPO-ANCA positivity (<i>p</i>&lt;0.0001), and less frequently pulmonary infiltrates (<i>p</i>=0.031). Median time from diagnosis-to-1<sup>st</sup> vasculitis relapse was 25 (11–60) months. During follow-up, their daily GC dose was lower than those with GC-dependent asthma/ENT manifestations, but similar to those in complete remission. At last follow-up, neurological sequelae tended to be more frequent (<i>p</i>=0.06). Finally, patients in complete remission were older (<i>p</i>&lt;0.0001), had more fever (<i>p</i>=0.03), less GC-treated asthma (<i>p</i>=0.002) and ENT manifestations at diagnosis (<i>p</i>=0.01), lower daily GC dose during follow-up (<i>p</i>&lt;0.0001), lower eosinophils count at 6 months (<i>p</i>=0.002) and less frequent sequelae (<i>p</i>=0.003). <h3>Conclusion</h3> 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. <h3>Disclosure of Interests</h3> Matthias Papo: None declared, Renato A. Sinico: None declared, Vítor 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, Cécile-Audrey Durel: None declared, Luc Mouthon: None declared, Xavier Puéchal: None declared, Loïc Guillevin: None declared, Giacomo Emmi: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared