Abstracts tagged "Eosinophilic Granulomatosus with Polyangiitis (Churg-Strauss)"

Abstract Number: 0677 • ACR Convergence 2023
Analysis of Clinical Outcomes in Eosinophilic Granulomatosis with Polyangiitis (EGPA) Treated with Mepolizumab over 2 Years for Remission Induction or Maintenance: A Single Center Experience in Japan
SOKO KAWASHIMA¹, Yoshinori Komagata¹, Mitsumasa Kishimoto² and Shinya Kaname¹, ¹Kyorin University School of Medicine, Tokyo, Japan, ²Kyorin University School of Medicine, Yokohoma, Japan
Background/Purpose: Contrary to Western countries, MPO-ANCA-associated vasculitis (MPO-AAV) is dominant in Japan or Asian countries. It is possible that therapeutic responses to EGPA with mepolizumab…
Abstract Number: 0678 • ACR Convergence 2023
Benralizumab in Eosinophilic Granulomatosis with Polyangiitis
Adrien Cottu¹, Matthieu Groh², Charlène Desaintjean³, Sylvain Marchand-Adam⁴, Loic Guillevin⁵, Xavier Puéchal⁶, Estibaliz Lazaro⁷, Maxime Samson⁸, Camille Taillé⁹, Cécile-Audrey Durel¹⁰, Elisabeth Diot¹¹, Sarah Nicolas¹¹, Laurent Guilleminault¹², Mikael Ebbo¹³, Pascal Cathébras¹⁴, Clairelyne Dupin⁹, Halil Yildiz¹⁵, Nabil Belfeki¹⁶, Gregory Pugnet¹⁷, Pierre Chauvin¹⁸, Stephane Jouneau¹⁹, François Lifermann²⁰, Jean-Philippe Martellosio²¹, Vincent Cottin²² and Benjamin Terrier²³, ¹Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ²National Referral Center for Hypereosinophilic Syndrome (CEREO), Hôpital Foch, Suresnes, France, ³Department of Respiratory Diseases, Hôpital Louis Pradel, Lyon, France, ⁴CHRU Tours, service de pneumologie et d'explorations fonctionnelles respiratoires, Tours, France, ⁵University Paris Descartes, Paris, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases, Paris, France, ⁷Bordeaux Hospital University, Pessac, France, ⁸Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ⁹AP-HP, Bichat Hospital, Reference Center for Rare Pulmonary Diseases and University of Paris Cité, Inserm 1152, Paris, France, ¹⁰CHU Lyon, Lyon, France, ¹¹Service de médecine interne et immunologie clinique, CHU Tours, Tours, France, ¹²Department of respiratory and allergic diseases, Toulouse University Hospital Center, Toulouse, France, ¹³Aix Marseille Univ, APHM, hôpital de la Timone, Internal Medicine Department, Marseille, France, ¹⁴Department of Internal medicine, Hôpital Nord, CHU St Etienne, Saint-Etienne, France, ¹⁵Saint Luc Hospital, Bruxelles, Belgium, ¹⁶Service de Médecine Interne et Immunologie Clinique. Groupe Hospitalier Sud Île de France, Melun, France, ¹⁷CHU Toulouse Rangueil Service de Medecine Interne et Immunologie Clinique, Toulouse, France, ¹⁸Department of Respiratory Diseases, Rennes University Hospital, Rennes, France, ¹⁹Amicus, Boca Raton, FL, ²⁰Department of Internal Medicine, Centre Hospitalier de Dax, Dax, France, ²¹Service de médecine interne, maladies infectieuses et tropicales, CHU de Poitiers, Poitiers, France, ²²Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University of Lyon, INRAE, Lyon, France, ²³Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: Gucocorticoid (GC)-dependant asthma and ENT exacerbations may persist in more than 80% of eosinophilic granulomatosis with polyangiitis (EGPA). The MIRRA trial demonstrated the efficacy…
Abstract Number: 0854 • ACR Convergence 2023
Long-term Efficacy of Remission-induction Regimens for Eosinophilic Granulomatosis with Polyangiitis
Martin Dutertre¹, Gregory Pugnet², Claire De Moreuil³, Bernard Bonnotte⁴, YGAL BENHAMOU⁵, Dominique Chauveau⁶, Elisabeth Diot⁷, Pierre Duffau⁸, Nicolas Limal⁹, Antoine Néel¹⁰, GEOFFREY URBANSKI¹¹, Noémie Jourde-Chiche¹², Nicolas MARTIN SILVA¹³, Francois Maurier¹⁴, Arsène Mekinian¹⁵, Nicolas Schleinitz¹⁶, Felix ackermann¹⁷, Anne-Laure Fauchais¹⁸, Antoine Froissart¹⁹, Thomas Le Gallou²⁰, Yurdagul Uzunhan²¹, Jean-Francois Viallard²², Alice Berezne²³, laurent chiche²⁴, Bruno Crestani²⁵, Guillaume Direz²⁶, Cecile-Audrey DUREL²⁷, Pascal Godmer²⁸, Jean-Emmanuel Kahn²⁹, Marc Lambert³⁰, Mathilde de Menthon¹, Thomas Quemeneur³¹, Jacques Cadranel¹, Pierre Charles³², Antoine Dossier¹, Loic Guillevin³³, Xavier Puéchal³⁴ and Benjamin Terrier³⁵, ¹AP-HP, Paris, France, ²CHU Toulouse Rangueil Service de Medecine Interne et Immunologie Clinique, Toulouse, France, ³CHU de Brest, Brest, France, ⁴Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ⁵rouen university hospital, Rouen, France, ⁶Hôpital Le Tripode, Bordeaux, France, ⁷Service de médecine interne et immunologie clinique, CHU Tours, Tours, France, ⁸CHU Bordeaux, Bordeaux, France, ⁹AP-HP, Créteil, France, ¹⁰CHU de Nantes, Nantes, France, ¹¹CHU Angers, Angers, France, ¹²AP-HM, Marseille, France, ¹³CHU Caen, Caen, France, ¹⁴Hôpitaux privés de Metz, Vaux / Frankreich, France, ¹⁵Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Paris, France, ¹⁶Aix Marseille university, AP-HM, Marseille, France, ¹⁷Hôpital Foch, Suresnes, France, ¹⁸Dupuytren Hospital, Limoges, France, ¹⁹CHI Créteil, Créteil, France, ²⁰CHU Rennes, Rennes, France, ²¹AP-HP, Bobigny, France, ²²CHU de Bordeaux, Hôpital Haut-Lévêque, Pessac, France, ²³CH Annecy, Annecy, France, ²⁴Hopital Europeen, Marseille, France, ²⁵Hopital Bichat, Paris University, Paris, France, ²⁶CH Le Mans, Le Mans, France, ²⁷Hospices Civils de Lyon, Lyon, France, ²⁸CH Bretagne Atlantique, Vannes, France, ²⁹AP-HP, Suresnes Cedex, France, ³⁰CHRU Lille, Lille, France, ³¹CH Valenciennes, Valenciennes, France, ³²Institut Mutualiste Montsouris, Service de Médecine Interne, Paris, France, ³³University Paris Descartes, Paris, France, ³⁴National Referral Center for Rare Systemic Autoimmune Diseases, Paris, France, ³⁵Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: The Rituximab in Eosinophilic Granulomatosis With Polyangiitis (REOVAS) trial compared rituximab (RTX) infusions to conventional strategy for remission-induction in eosinophilic granulomatosis with polyangiitis (EGPA).…
Abstract Number: 0856 • ACR Convergence 2023
Dupilumab for Relapsing or Refractory Eosinophilic Granulomatosis with Polyangiitis: A European Retrospective Study
Berengere Molina¹, Roberto Padoan², Maria Letizia Urban³, Pavel Novikov⁴, Marco Caminati⁵, Camille Taillé⁶, Antoine Néel⁷, Laurence Bouillet⁸, Paolo Fraticelli⁹, Nicolas Schleinitz¹⁰, Christine Christides¹¹, Laura Moi¹², Bertrand Godeau¹³, Ann Knight¹⁴, Jan Walter Schroeder¹⁵, Sylvain Marchand-Adam¹⁶, Helder Gil¹⁷, Vincent Cottin¹⁸, Cécile-Audrey Durel¹⁹, Elena Gelain²⁰, Boris Lerais²¹, Marc Ruivard²², Matthieu Groh²³, Maxime Samson²⁴, Luca Moroni²⁵, Jens Thiel²⁶, Anna Kernder²⁷, Jan Willem Cohen Tervaert²⁸, Giulia Costanzo²⁹, Marco Folci³⁰, Sonia Rizzello³¹, Pascal Cohen³², Giacomo Emmi³³ and Benjamin Terrier³⁴, ¹Cochin Hospital, Paris, France, ²Department of Medicine DIMED, University of Padova, Padova, Italy, ³Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁴Sechenov First Moscow State Medical University, Moscow, Russia, ⁵University of Verona, Verona, Italy, ⁶AP-HP, Bichat Hospital, Reference Center for Rare Pulmonary Diseases and University of Paris Cité, Inserm 1152, Paris, France, ⁷CHU Nantes, Nantes, France, ⁸Internal medicine department, Grenoble University Hospital, Grenoble, France, ⁹University Hospital Ospedali Riuniti, Ancona, Italy, ¹⁰Aix Marseille university, AP-HM, Marseille, France, ¹¹Avignon Hospital, Avignon, France, ¹²Valais Hospital, Sion, Switzerland, ¹³CHU Henri Mondor, Créteil, France, ¹⁴Uppsala University, Uppsala, Sweden, ¹⁵ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy, ¹⁶CHRU Tours, service de pneumologie et d'explorations fonctionnelles respiratoires, Tours, France, ¹⁷CHU Besancon, Besançon, France, ¹⁸Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University of Lyon, INRAE, Lyon, France, ¹⁹CHU Lyon, Lyon, France, ²⁰Meyer Children's Hospital, Florence, Italy, ²¹Brest University Hospital, Brest, France, ²²CHU Clermont Ferrand, Clermont Ferrand, France, ²³National Referral Center for Hypereosinophilic Syndrome (CEREO), Hôpital Foch, Suresnes, France, ²⁴Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ²⁵San Raffaele Scientific Institute, Milan, Italy, ²⁶University Hospital Freiburg, Freiburg, Germany, ²⁷Heinrich-Heine-University Düsseldorf, Dusseldorf, Germany, ²⁸University of Alberta, Edmonton, AB, Canada, ²⁹University of Cagliari, Monserrato, Italy, ³⁰Fondazione Poliambulanza Istituto Ospedaliero, Brescia, Italy, ³¹Careggi University Hospital, Florence, Italy, ³²CHU Cochin, Paris, France, ³³University of Florence, Florence, Italy, ³⁴Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitides characterized by asthma, blood and tissue eosinophilia and systemic manifestations. Glucocorticoids (GCs)-dependent asthma and/or disabling…
Abstract Number: 1546 • ACR Convergence 2023
Eosinophilic Granulomatosis with Polyangiitis: Clinical Suspicion Red Flags Identification by a Systematic Literature Review and Multidisciplinary Expert Consensus
Ricardo Blanco¹, Iñigo Rúa-Figueroa², Roser Solans³, Maria C Cid⁴, Marina Blanco⁵, Ismael Garcia Moguel⁶, Francisco Perez Grimaldi⁷, Ana Noblejas⁸, Moises Labrador³, Cristian Domingo⁹, Georgina espigol¹⁰, Fernando Sanchez Toril¹¹, Francisco Ortiz-Sanjuán¹², Ebymar Arismendi¹³ and Jose María Alvaro-Gracias¹⁴, ¹Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ²Rheumatology, Hospital de Gran Canaria Doctor Negrin, Las Palmas de Gran Canaria, Spain, ³Internal Medicine Department, H. Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain, ⁴Hospital Clinic Barcelona, Barcelona, Spain, ⁵Pneumology Department, Hospital Universitario de A Coruña, Madrid, Spain, ⁶Allergy Department, Hospital Universitario 12 de Octubre, Madrid, Spain; Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), Madrid, Spain, ⁷Pneumology Department, H. University of Jerez, Cádiz, Spain, ⁸Internal Medicine Department, Hospital Universitario La Paz, Madrid, Spain, ⁹Pneumology Department, Corporació Sanitaria Parc Taulí, Universitat Autònoma de Barcelona, Barcelona, Spain, ¹⁰Autoimmune Diseases Department, Hospital Clínic, FCRB-IDIBAPS, Universitat de Barcelona, Barcelona, Spain, ¹¹Pneumology Department, Hospital Arnau Vilanova, Valencia, Spain;, Valencia, Spain, ¹²Hospital Universitario y Politécnico La Fe, Valencia, Spain, ¹³Pneumology Department, Hospital Clínic de Barcelona, Barcelona, Spain, Universitat de Barcelona, Spain and CIBERES, Barcelona, Spain, ¹⁴Hospital General Universitario Gregorio Marañon, Madrid, Spain
Background/Purpose: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare ANCA-associated vasculitis, characterized histologically by eosinophilic tissue infiltration, necrotizing vasculitis, and eosinophil-rich granulomatous inflammation. The diagnosis…
Abstract Number: 1547 • ACR Convergence 2023
Disease Burden of Eosinophilic Granulomatosis with Polyangiitis (EGPA): A Retrospective Analysis of US Health Insurance Claims Data
Paul Dolin¹, Danuta Kielar¹, Anat Shavit¹, Karina Keogh², Jennifer Rowell¹, Chris Edmonds³, Juliana Meyers⁴, Elizabeth Esterberg⁴, Tram Nham⁴ and Stephanie Chen⁵, ¹AstraZeneca, Cambridge, United Kingdom, ²Mayo Clinic, Rochester, MN, ³AstraZeneca, Gaithersburg, MD, ⁴RTI Health Solutions, Research Triangle Park, NC, ⁵BioPharmaceuticals Medical, AstraZeneca, Gaithersburg, MD
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare necrotizing small-to-medium vessel vasculitis with a relapsing-remitting course involving prodromal (various allergic reactions), eosinophilic (infiltration into…
Abstract Number: 0432 • ACR Convergence 2022
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
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…
Abstract Number: 0453 • ACR Convergence 2022
Validation of the 2022 – American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria in Patients with ANCA Associated Vasculitis
Aman Sharma¹, Prateek Deo¹, Shankar Naidu², Joydeep Samanta³, Sakshi Mittal³, Kusum Sharma³, Ritambhra Nada³, Varun Dhir⁴, sanjay jain² and Ranjana Minz², ¹PGIMER, Chandigarh, India, Chandigarh, India, ²Postgraduate Institute of Medical Education and Research, Chandigarh, India, ³PGIMER, Chandigarh, India, ⁴PGIMER, CHD, INDIA, Chandigarh, India
Background/Purpose: The purpose of this study was to validate the recently published 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for…
Abstract Number: 0455 • ACR Convergence 2022
Performance of 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria in Turkish Vasculitis Patients
Ummugulsum Gazel¹, Sevinc Can Sandikci², Ahmet Omma³, Sinem Burcu Kocaer⁴, Fatoş Onen⁵, Onay Gercik⁶, Servet Akar⁷, Kerem Abacar⁸, Ebru Asicioglu⁹, Serhan Tuglular⁹, Fatma Alibaz-Oner⁸ and Haner Direskeneli¹⁰, ¹University of Ottawa, Rheumatology, Ottawa, Canada, Ottawa, ON, Canada, ²Ankara Numune Education and Research Hospital, Rheumatology, Ankara, Turkey, ³Ankara Numune Education and Research Hospital, Rheumatology, Ankara, Turkey, Istanbul, Turkey, ⁴Dokuz Eylul University, Faculty of Medicine, Rheumatology, Izmir, Turkey, ⁵Dokuz Eylul University, Faculty of Medicine, Rheumatology, İzmir, Turkey, ⁶Izmir Tepecik Research Hospital, Izmir, Turkey, ⁷Izmir Katip Celebi University School of Medicine, Izmir, Turkey, ⁸Marmara University, Rheumatology, Istanbul, Turkey, ⁹Marmara University, Faculty of Medicine, Nephrology, Istanbul, Turkey, ¹⁰Marmara University, Rheumatology, Istanbul, Istanbul, Turkey
Background/Purpose: The American College of Rheumatology (ACR)-1990 criteria is the most used method for the classification of ANCA Associated Vasculitis (AAV). However, with ACR-1990 criteria,…
Abstract Number: 0457 • ACR Convergence 2022
A Systematic Literature Review Informing the 2022 Update of the EULAR Recommendations for the Management of ANCA-associated Vasculitis
Jan Schirmer¹, Beatriz Sanchez-Alamo², Sara Monti³, Bernhard Hellmich⁴, David Jayne⁵ and Gunnar Tomasson⁶, ¹University Medical Center Schleswig-Holstein, Kiel, Germany, ²Lund University, Skåne University Hospital, Lund, Sweden, ³Rheumatology, Fondazione IRCCS Policlinico S. Matteo, University of Pavia, Pavia, Italy, ⁴Medius Kliniken, University of Tübingen, Kirchheim Teck, Germany, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶Centre for Rheumatology Research, University Hospital Reykjavik, Reykjavik, Iceland
Background/Purpose: Since the publication of the 2016 European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of ANCA-associated vasculitis (AAV), the publication of…
Abstract Number: 1074 • ACR Convergence 2022
Safety and Efficacy of Dupilumab in Patients with Relapsing and/or Refractory Eosinophilic Granulomatosis with Polyangiitis
Berengere Molina¹, Maria Letizia Urban², Roberto Padoan³, Pavel Novikov⁴, Camille Taillé⁵, Marco Caminati⁶, Christine Christides⁷, nicolas schleinitz⁸, Laura Moi⁹, Vincent Cottin¹⁰, bertrand godeau¹¹, Laurence Bouillet¹², Paolo Fraticelli¹³, Ann Knight¹⁴, Jan Walter Schroeder¹⁵, Sylvain Marchand-Adam¹⁶, Helder Gil¹⁷, Elena Gelain¹⁸, Giacomo Emmi² and Benjamin Terrier¹⁹, ¹Department of Internal Medicine, Cochin Hospital, Paris, France, ²Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ³University of Padova, Padova, Italy, ⁴Tareev’s Clinic, Sechenov First Moscow State Medical University, Moscow, Russia, ⁵Pulmonology department, Bichat Hospital, Paris, Ile-de-France, France, ⁶Verona University Hospital, University of Verona, Verona, Italy, ⁷Avignon hospital, Avignon, France, ⁸Aix Marseille university, AP-HM, Marseille, France, ⁹Centre hospitalier universitaire vaudois, Lausanne, Lausanne, Switzerland, ¹⁰Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, University of Lyon, INRAE, Lyon, France, ¹¹Department of Internal Medicine, National Referral Center for Adult's Immune Cytopenias, Mondor Hospital, Créteil, France, ¹²Grenoble University Hospital, Grenoble, France, ¹³Department of Internal Medicine, Ospedali Riuniti di Ancona, Ancona, Italy, ¹⁴Akademiska sjukhuset, Uppsala, Sweden, ¹⁵ASST GOM Niguarda, Milano, Italy, ¹⁶Tours University Hospital, Tours, France, ¹⁷Besancon University Hospital, Besançon, France, ¹⁸Meyer Children’s University Hospital of Firenze, Florence, Italy, ¹⁹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitides characterized by asthma, blood and tissue eosinophilia and systemic manifestations. Glucocorticosteroids (GCs)-dependent asthma and/or disabling…
Abstract Number: 1075 • ACR Convergence 2022
Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis and a Vasculitic Phenotype
Benjamin Terrier¹, David Jayne², Bernhard Hellmich³, Jane Bentley⁴, Jonathan Steinfeld⁵, Steven Yancey⁶, Namhee Kwon⁷, Praveen Akuthota⁸, Paneez Khoury⁹, Lee Baylis¹⁰ and Michael Wechsler¹¹, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²University of Cambridge, Cambridge, United Kingdom, ³Klinik für Innere Medizin, Rheumatologie & Immunologie, Medius Kliniken, Universität Tübingen, Plochingen, Germany, ⁴Clinical Statistics, GlaxoSmithKline, Brentford, United Kingdom, ⁵Clinical Sciences, Respiratory, GSK (affiliation at time of research), Philadelphia, PA, ⁶Respiratory Therapeutic Area, GSK (affiliation at the time of research), Research Triangle Park, NC, ⁷Clinical Sciences, Respiratory, GSK, Brentford, United Kingdom, ⁸Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of California, San Diego, CA, ⁹Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ¹⁰Global Medical Affairs, GlaxoSmithKline, Durham, NC, ¹¹Department of Medicine, National Jewish Health, Denver, CO
Background/Purpose: Patients with eosinophilic granulomatosis with polyangiitis (EGPA) can have vasculitic or eosinophilic phenotypes. The MIRRA study demonstrated that patients with EGPA spent more time…
Abstract Number: 1767 • ACR Convergence 2022
Epidemiology of Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Hypereosinophilic Syndrome (HES) in Germany: A Claims Database Study
Bernhard Hellmich¹, Konstantin Neukirch², Marco Lukas², Martin Wernitz², Dominik Beier³ and Dennis Häckl⁴, ¹Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken, University of Tübingen, Kirchheim Teck, Germany, ²GlaxoSmithKline GmbH & Co. KG, Munich, Germany, ³InGef - Institute for Applied Health Research Berlin GmbH, Berlin, Germany, ⁴WIG2 GmbH, Leipzig, Germany
Background/Purpose: EGPA and HES are rare multisystemic diseases associated with eosinophilia. Robust data on the epidemiology and treatment of HES and EGPA are scarce. The…
Abstract Number: 0125 • ACR Convergence 2022
Baseline Characteristics in Patients with Eosinophilic Granulomatosis with Polyangiitis in the U.S. Initiating Mepolizumab
Jared Silver¹, Arijita Deb², Elizabeth Packnett³, Donna McMorrow³, Cynthia Morrow³ and Michael Bogart¹, ¹GlaxoSmithKline, Research Triangle Park, NC, ²GlaxoSmithKline, Upper Providence, PA, ³IBM Watson Health, Cambridge, MA
Background/Purpose: EGPA is a multisystem disorder often characterized clinically by asthma, chronic rhinosinusitis, and prominent eosinophilia, and histopathologically by eosinophil-rich, necrotizing , granulomatous vasculitis of…
Abstract Number: L21 • ACR Convergence 2021
Rituximab versus Conventional Therapeutic Strategy for Remission Induction in Eosinophilic Granulomatosis with Polyangiitis: A Double-blind, Randomized, Controlled Trial
Benjamin Terrier¹, Gregory Pugnet², Claire de Moreuil³, Bernard Bonnotte⁴, Ygal Benhamou⁵, Elisabeth Diot⁶, Dominique Chauveau², Pierre Duffau⁷, Nicolas Limal⁸, Antoine Neel⁹, Geoffrey Urbanski¹⁰, Noemie Jourde-Chiche¹¹, Nicolas Martin-Silva¹², Francois Maurier¹³, Arsene Mekinian¹⁴, Nicolas Schleinitz¹¹, Felix Ackermann¹⁵, Anne-Laure Fauchais¹⁶, Antoine Froissart¹⁷, Thomas Le Gallou¹⁸, Yurdagul Uzunhan¹⁹, Jean-Francois Viallard⁷, Alice Berezne²⁰, Laurent Chiche²¹, Bruno Crestani¹⁴, Guillaume Direz²², Cecile-Audrey Durel²³, Pascal Godmer²⁴, Jean-Emmanuel Kahn²⁵, Marc Lambert²⁶, Thomas Quemeneur²⁷, Jacques Cadranel¹⁴, Pierre Charles²⁸, Antoine Dossier¹⁴, Lea Jilet²⁹, Loic Guillevin¹⁴, Hendy Abdoul²⁹ and Xavier Puechal¹⁴, ¹Cochin Hospital, Paris, France, ²CHU, Toulouse, France, ³CHU, Brest, France, ⁴CHU, Dijon, France, ⁵CHU, Rouen, France, ⁶CHU, Tours, France, ⁷CHU, Bordeaux, France, ⁸CHU, Creteil, France, ⁹CHU, Nantes, France, ¹⁰CHU, Angers, France, ¹¹CHU, Marseille, France, ¹²CHU, Caen, France, ¹³CH, Metz, France, ¹⁴CHU, Paris, France, ¹⁵Hopital Foch, Suresnes, France, ¹⁶CHU, Limoges, France, ¹⁷CHIC, Creteil, France, ¹⁸CHU, Rennes, France, ¹⁹CHU, Bobigny, France, ²⁰CH, Annecy, France, ²¹CH, Marseille, France, ²²CH, Le Mans, France, ²³CHU, Lyon, France, ²⁴CH, Vannes, France, ²⁵CHU, Boulogne, France, ²⁶CHRU, Lille, France, ²⁷CH, Valenciennes, France, ²⁸CH, Paris, France, ²⁹URC, Paris, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophilic ANCA-associated vasculitis.Glucocorticoids, alone or in combination with cyclophosphamide in severe forms, induce remission in most patients…

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