Predictive Factors of Eosinophilic Granulomatosis with Polyangiitis Long-term Evolution: Data from a European Cohort

Matthias Papo¹, Pauline Martinot², Renato A. Sinico³, Vitor Teixeira⁴, Nils Venhoff⁵, Maria-Letizia Urban⁶, Juliane Mahrhold⁷, Francesco Locatelli⁸, Giulia Cassone⁹, Franco Schiavon¹⁰, Benjamin Seeliger¹¹, Thomas Neumann¹², Claus Kroegel¹³, Matthieu Groh¹⁴, Chiara Marvisi¹⁵, Maxima Samson¹⁶, Thomas Barba¹⁷, David Jayne¹⁸, Arianna Troilo¹⁹, Jens Thiel²⁰, Bernhard Hellmich²¹, Sara Monti²², Carlomaurizio Montecucco⁸, Carlo Salvarani²³, Jean-Emmanuel Kahn²⁴, Cecile-Audrey DUREL²⁵, Luc Mouthon²⁶, Loïc Guillevin²⁶, Giacomo Emmi²⁷, Augusto Vaglio²⁸, Raphaël Porcher²⁹ and Benjamin Terrier²⁶, ¹Service De Médecine Interne - Hôpital Cochin, Paris, France, ²Centre of Research in Epidemiology and Statistics, Université de Paris, Paris, Île-de-France, France, Paris, ³Department of Medicine and Surgery, Università degli Studi di Milano – Bicocca, Italy, Milano, Italy, ⁴Hospital de Faro, CHUA, Lisbon, Portugal, ⁵Department of Rheumatology and Clinical Immunology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany, ⁶Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy, ⁷Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken, University of Tübingen, Kirchheim Teck, Germany, ⁸Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy, ⁹Università di Modena e Reggio Emilia, Modena, Italy, ¹⁰Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy, ¹¹Department of Respiratory Medicine, Hannover Medical School, Wermsdorf, Germany, ¹²Kantonsspital St. Gallen, St. Gallen, Switzerland, ¹³Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany, ¹⁴Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, ¹⁵Università di Modena e Reggio Emilia and National Institute of Arthritis & Musculoskeletal & Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁶Dijon University Hospital, Dijon, France, ¹⁷Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, ¹⁸University of Cambridge, Cambridge, United Kingdom, ¹⁹Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany, ²⁰University Hospital Freiburg, Freiburg, Germany, ²¹Klinik für Innere Medizin, Rheumatologie & Immunologie, Medius Kliniken, Universität Tübingen, Plochingen, Germany, ²²Rheumatology, Fondazione IRCCS Policlinico S. Matteo, University of Pavia, Pavia, Italy, ²³Azienda USL -IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Reggio Emilia, Italy, ²⁴Service de médecine interne - Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France, ²⁵CHU Lyon, Lyon, France, ²⁶National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²⁷Azienda Ospedaliero Universitaria Careggi, Firenze, Italy, ²⁸Azienda Ospedaliero Universitaria Meyer, Parna, Italy, ²⁹Université Paris Cité, Hôtel-Dieu, Paris, France

Meeting: ACR Convergence 2022

Keywords: ANCA associated vasculitis, Eosinophilic Granulomatosus with Polyangiitis (Churg-Strauss), prognostic factors

Session Information

Date: Saturday, November 12, 2022

Title: Vasculitis – ANCA-Associated Poster I: Epidemiology, Outcomes, and Classification

Session Type: Poster Session A

Session Time: 1:00PM-3:00PM

Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) frequently control the disease, but relapses and/or GC-dependency are 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. Our objective was to build models to predict the evolution towards a relapse of vasculitis or towards a GC-dependent asthma and/or ENT manifestations.

Methods: We set up a multicenter European retrospective study of EGPA patients. EGPA diagnosis was made according to ACR and/or MIRRA criteria. We developed a multivariable prediction model using the PMSAMPSIZE algorithm: one model for the vasculitis relapse risk during follow-up (excluding asthma and/or ENT manifestations) and one model for the risk of GC-dependent asthma and/or ENT manifestations at two years of follow-up.

Results: We included 809 patients. Patients’ middle age was 52 (+/- 14.8) years, and median follow-up was of 72 months (IQR 37/115). During follow-up, 228 experienced at least one vasculitis relapse, with a cumulative incidence at 12 years of 41.2% (95% CI 36.3-46.8). At two years of follow-up, 66.4% of patients had GC-dependent asthma and/or ENT manifestations.

First, a model predicting the risk of vasculitis relapse included the following variables: age at diagnosis (nonlinear effect), GC-dependent asthma before EGPA diagnosis (HR 1.57 [1.24-2.55]), arthralgia (HR 1.27 [1.01-1.60]), myocarditis (HR 1.74 [1.16-2.62]), peripheral neuropathy (HR 1.39 [1.09-1.79]), MPO-ANCA (HR 1.56 [1.22-2.01]) and baseline eosinophils count (nonlinear effect]). Final model allowed to modelize a nomogram and calculate a score establishing the vasculitis relapse risk at 5 and 12 years of follow-up.

Second, a predicting the risk of GC-dependent asthma and/or ENT manifestations at 2 years included the following variables: age at diagnosis (per year, OR 0.98 [0.97-0.99]), GC-dependent asthma at EGPA diagnosis (OR 1.50 [1.02-2-20]), history of chronic sinusitis before EGPA diagnosis (OR 1.78 [1.27-2.48]), and baseline eosinophils count (per 10.10⁹/L, OR 0.70 [0.49-1.00]). Final model allowed to modelize a nomogram and calculate a score establishing the risk of GC-dependent asthma and/or ENT manifestations at 2 years.

Conclusion: Through a large retrospective European EGPA cohort, we developed models predicting the vasculitis relapse risk and the risk of GC-dependent asthma and/or ENT manifestations at 2 years. These models must be validated in validation cohorts and could be used to guide the use of biotherapies targeting B cells or eosinophils.

Disclosures: M. Papo, None; P. Martinot, None; R. Sinico, None; V. Teixeira, None; N. Venhoff, None; M. Urban, None; J. Mahrhold, None; F. Locatelli, None; G. Cassone, None; F. Schiavon, None; B. Seeliger, None; T. Neumann, None; C. Kroegel, None; M. Groh, None; C. Marvisi, None; M. Samson, None; T. Barba, None; D. Jayne, Aurinia, AstraZeneca, GlaxoSmithKline (GSK), Roche/Genentech, Vifor, Bristol-Myers Squibb(BMS), Chemocentryx, Novartis, Takeda, Boehringer-Ingelheim, Otsuka, UCB, Amgen, Kessai; A. Troilo, None; J. Thiel, None; B. Hellmich, AbbVie, Amgen, AstraZeneca, Bristol-Myers Squibb (BMS), Boehringer-Ingelheim, Chugai, GlaxoSmithKline (GSK), InflaRx, Janssen, MSD, Novartis, Pfizer, Phadia, Roche, Vifor; S. Monti, None; C. Montecucco, AbbVie/Abbott, Bristol-Myers Squibb(BMS), Eli Lilly, Gilead, Roche, Pfizer, Sanofi; C. Salvarani, None; J. Kahn, None; C. DUREL, None; L. Mouthon, Boehringer-Ingelheim, LFB; L. Guillevin, Roche; G. Emmi, None; A. Vaglio, None; R. Porcher, None; B. Terrier, AstraZeneca, GlaxoSmithKline, Bristol-Myers Squibb(BMS), Eli Lilly, LFB, Boehinger Ingelheim, Vifor Pharma, Pfizer, Roche.

To cite this abstract in AMA style:

Papo M, Martinot P, Sinico R, Teixeira V, Venhoff N, Urban M, Mahrhold J, Locatelli F, Cassone G, Schiavon F, Seeliger B, Neumann T, Kroegel C, Groh M, Marvisi C, Samson M, Barba T, Jayne D, Troilo A, Thiel J, Hellmich B, Monti S, Montecucco C, Salvarani C, Kahn J, DUREL C, Mouthon L, Guillevin L, Emmi G, Vaglio A, Porcher R, Terrier B. Predictive Factors of Eosinophilic Granulomatosis with Polyangiitis Long-term Evolution: Data from a European Cohort [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/predictive-factors-of-eosinophilic-granulomatosis-with-polyangiitis-long-term-evolution-data-from-a-european-cohort/. Accessed .

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