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

Abstract Number: 1592 • ACR Convergence 2024
Overlapping Forms of Eosinophilic Granulomatosis with Polyangiitis and Granulomatosis with Polyangiitis: Presentation, Management and Outcomes
Federica Pallotti¹, Camille Mettler², Roberto Padoan³, Francesca Regola⁴, Franco Franceschini⁵, Sergey Moiseev⁶, Pavel Novikov⁷, Mario Andrea Piga⁸, Gianluca Moroncini⁹, Silke Brix¹⁰, Abdul Hadi Kafagi¹¹, Samuel Deshayes¹², Achille Aouba¹², Julien Campagne¹³, Paolo Delvino¹⁴, Jan Willem Cohen Tervaert¹⁵, Luisa Brussino¹⁶, Martin Michaud¹⁷, Nils Venhoff¹⁸, Federico Alberici¹⁹, Claudia Iannone²⁰, Sophie Rosenstingl²¹, Marin Moutel²², Jean-Marc Galempoix²³, Vincent Cottin²⁴, Clara Jaccard²⁵, Diane Riehl²⁶, Paul Legendre²⁷, Anne-Claire Billet²⁸, Paola Parronchi²⁹, Luca Quartuccio³⁰, Vítor Silvestre Teixeira³¹, Allyson Egan³², David Jayne³², Enrico Tombetti³³, Marco Caminati³⁴, Christian Pagnoux³⁵, Alexis Régent³⁶, Marc Ruivard³⁷, Loïc Guillevin³⁸, Xavier Puéchal³⁶ and Benjamin Terrier³⁹, and the French Vasculities Study Group and European EGPA Study Group, ¹Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France, ²Département de Médecine Interne, Centre de Référence National pour les maladies auto-immunes systémiques rares, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France, ³Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ⁴Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili Brescia and University of Brescia, Brescia, Italy, ⁵Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ⁶Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia, ⁷Sechenov First Moscow State Medical University, Moscow, Russia, ⁸Postgraduate School of Allergy and Clinical Immunology, Università Politecnica delle Marche, Ancona, Italy, ⁹Department of Clinical and Molecular Sciences, Marche Polytechnic University & Department of Internal Medicine, Marche University Hospital, Ancona, Italy, ¹⁰Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, The University of Manchester, Manchester, United Kingdom, ¹¹Division of Cardiovascular Sciences, School of Medical Sciences, University of Manchester, Manchester, United Kingdom, ¹²Service d'immunologie clinique-médecine interne, CHU de Caen Normandie, Caen, France, ¹³Hôpital Robert Schuman - Competence center for autoimmune diseases, Internal Medicine, Metz, France, ¹⁴University of Milano-Bicocca, Milan, Milan, Italy, ¹⁵University of Alberta, Edmonton, Canada, ¹⁶SSDDU Immunologia Clinica ed Allergologia, AO Mauriziano, Turin, Italy, ¹⁷Department of Internal Medicine, Clinique Saint-Exupery, Toulouse, France., Toulouse, France, ¹⁸Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, ¹⁹Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy, ²⁰Department of Rheumatology, University of Milan, and Rheumatology Department, ASST Pini-CTO, Milan, Italy, ²¹Service Médecine Interne, Centre hospitalier intercommunal Compiègne Noyon, Compiègne Noyon, France, ²²Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims University Hospital, Reims, France, ²³J.M. Galempoix, MD, Department of Internal Medicine, Nord Ardennes Hospital, Charleville-Mézières, France, ²⁴Hôpital Louis Pradel, Centre de Référence des Maladies Pulmonaires Rares, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France, Lyon, France, ²⁵Gaston Bourret Hospital, Nouméa, New Caledonia, ²⁶Centre hospitalier intercommunal Toulon- La Seyne Sur Mer, Toulon, France, ²⁷Service Médecine Interne et Polyvalente, Centre Hospitaliers Le Mans, Le Mans, France, ²⁸Department of Internal Medicine, Édouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France, ²⁹University of Florence, Florence, Florence, Italy, ³⁰Division of Rheumatology, Department of Medicine, University of Udine, Udine, Italy, ³¹Department of Rheumatology, Faro Hospital, Algarve, Portugal, ³²University of Cambridge, Cambridge, United Kingdom, ³³Internal Medicine and Rheumatology, Department of Biomedical and Clinical Sciences, Sacco and Fatebenefratelli Hospitals, Milan, Italy, Milan, Italy, ³⁴Department of Medicine, Asthma, Allergy and Clinical Immunology Section, University of Verona, Verona, Italy, ³⁵Mount Sinai Hospital, Toronto, ON, Canada, ³⁶National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ³⁷Internal Medicine Department, Estaing University Hospital, CHU Clermont-Ferrand, Clermonnt-Ferrand, Italy, ³⁸National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ³⁹Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: ANCA-associated vasculitis (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Although these entities are often easily distinguished in…
Abstract Number: 2491 • ACR Convergence 2024
Treatment Outcomes of Eosinophilic Granulomatosis with Polyangiitis (EGPA):A Retrospective Analysis of US Health Insurance Claims Data
Paul Dolin¹, Anat Shavit¹, Karina A. Keogh², Jennifer Rowell³, Chris Edmonds⁴, Danuta Kielar⁵, Juliana Meyers⁶, Elizabeth Esterberg⁶, Tram Nham⁶ and Stephanie Y. Chen⁷, ¹BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ²Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic,, Rochester, MN, ³Market Access and Pricing, AstraZeneca, Cambridge, England, United Kingdom, ⁴Market Access and Pricing, AstraZeneca, Gaithersburg, MD, ⁵Biopharmaceuticals Medicine, AstraZeneca, Cambridge, United Kingdom, ⁶RTI Health Solutions, Research Triangle Park, NC, ⁷BioPharmaceuticals Medical, AstraZeneca, Gaithersburg, MD
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis disorder characterized by asthma, eosinophilia and the presence of eosinophilic inflammation. Patients with EGPA are…
Abstract Number: 1593 • ACR Convergence 2024
Efficacy of Increasing the Dose of Mepolizumab in Eosinophilic Granulomatosis with Polyangiitis
Federica Pallotti¹, Philippe Bonniaud², Perrine SMETS³, stephanie Habib⁴, Amandine Perier⁵, Alban Deroux⁶, Julie Mankikian⁷, Antoine Neel⁸, Clémentine Rousselin⁹, Raphaele Seror¹⁰, Camille Taille¹¹, Etienne Crickx¹², Candice La Croix¹³, Philippe Blanche¹⁴, Loïc Guillevin¹⁵, Xavier Puéchal¹⁶ and Benjamin Terrier¹⁷, and French Vasculitis Study Group, ¹Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France, ²Centre de Référence Constitutif des Maladies Pulmonaires Rares de l'Adulte, Service de Pneumologie et Soins Intensifs Respiratoires, Centre Hospitalo-Universitaire de Dijon-Bourgogne, Dijon, France, ³Clermont Ferrand University Hospital - National reference center for autoimmune disease, Internal Medicine, Clermont-Ferrand, France, ⁴Service de Pneumologie, Hôpital Cochin, APHP Centre et Université Paris Cité, Paris, France, ⁵CH de Niort, Niort, France, ⁶Clinique Universitaire de Médecine Interne, Department of Internal Medicine, Grenoble University Hospital, Grenoble, France, ⁷CHRU Tours, Service de Pneumologie et d'Explorations Fonctionnelles Respiratoires, Tours, France, ⁸Department of Internal Medicine, CHU Nantes, Nantes, France, ⁹Service de médecine interne et néphrologie, centre hospitalier de Valenciennes, Valenciennes, France, ¹⁰Service de Rhumatologie, Hôpital Bicêtre, AP-HP, le Kremlin Bicetre, Ile-de-France, France, ¹¹Bichat Hospital, Paris, France, ¹²Service de Médecine Interne, Centre national de référence des cytopénies auto-immunes de l'adulte, Hôpital Henri Mondor, APHP, Paris, France, ¹³Department of Otolaryngology, Hôpital Cochin, APHP, Paris, France, ¹⁴Department of Internal Medicine, National Referrence Center for Systemic and Autoimmune Diseases, Hôpital Cochin, APHP, Paris, France, ¹⁵National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ¹⁶National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ¹⁷Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) patients frequently develop glucocorticoid (GC)-dependent asthma and/or ENT manifestations, leading to long-term GC requirement and side effects. Mepolizumab, an…
Abstract Number: 2691 • ACR Convergence 2024
Transcriptomic Changes in CD4+ T Lymphocytes in Eosinophilic Granulomatosis with Polyangiitis
Roberto Ríos-Garcés¹, Núria Farran¹, Salvador Naranjo-Suarez², Roser Alba-Rovira¹, Sergio Prieto-González³, Itziar Tavera-Bahillo¹, Roser Solans-Laqué⁴, Ebymar Arismendi⁵, Marc Corbera-Bellalta¹, Javi Marco-Hernández¹, Farah Kamberovic¹, Nina Visocnik¹, Maria Cid⁶ and Georgina Espígol-Frigolé¹, ¹Vasculitis Research Group, Autoimmune Diseases Department, IDIBAPS, Hospital Clinic, University of Barcelona, Barcelona, Spain, ²Angiogenesis in Liver Disease Research Group, IDIBAPS, Hospital Clinic, University of Barcelona, Barcelona, Spain, ³Vasculitis Research Group, Autoimmune Diseases Department, IDIBAPS, Hospital Clinic, University of Barcelona, bar, Spain, ⁴Internal Medicine Service, Vall d'Hebron Hospital University and Campus, Barcelona, Spain, ⁵Pneumology Department, Hospital Clinic, IDIBAPS, University of Barcelona, CIBERES, Barcelona, Spain, ⁶Hospital Clinic Barcelona. University of Barcelona, Barcelona, Spain
Background/Purpose: Eosinophilic Granulomatosis with polyangiitis (EGPA) is a rare systemic autoimmune disorder, included within the ANCA-associated vasculitis. It is characterized by a diverse clinical profile,…
Abstract Number: 1594 • ACR Convergence 2024
Response to Mepolizumab Therapy in a Single-center Cohort of Eosinophilic Granulomatosis with Polyangiitis Patients: Characterization of Cytokine/Chemokine Pattern and Phenotypic Stratification
Michele Moretti¹, Francesco Ferro², Irene Martelli³, Francesca Pistone³, Giulia Greco³, Federica Di Cianni⁴, Nazzareno Italiano⁵, ROSARIA TALARICO⁴, Veronica Seccia⁶, Chiara Baldini⁷, Ilaria Puxeddu³ and Marta Mosca⁷, ¹University of Pisa, Pisa, Pisa, Italy, ²Clinical and Experimental Medicine Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Pisa, Italy, ³Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy, ⁴University of Pisa, Pisa, Italy, ⁵University of Pisa, Rovigo, Italy, ⁶Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy, ⁷University of Pisa, Department of Clinical and Experimental Medicine - Rheumatology Unit, Pisa, Italy
Background/Purpose: The introduction of Mepolizumab treatment has revolutionized the therapy of EGPA and has promoted the search for biomarkers predictive of response to treatment. However,…
Abstract Number: 1596 • ACR Convergence 2024
Efficacy of Eosinophil-Targeting Therapies According to Disease Severity in Patients with Eosinophilic Granulomatosis with Polyangiitis
Bernhard Hellmich¹, Peter Merkel², David Jayne³, Benjamin Terrier⁴, Florence Roufosse⁵, Parameswaran Nair⁶, Nader Khalidi⁶, David J. Jackson⁷, Shunsuke Furuta⁸, Lena Börjesson Sjö⁹, Sofia Necander⁹, Anat Shavit¹⁰, Claire Walton¹¹ and Michael Wechsler¹², ¹Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, ²University of Pennsylvania, Philadelphia, PA, ³University of Cambridge, Cambridge, United Kingdom, ⁴Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, ⁵Department of Internal Medicine, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium, ⁶McMaster University and St Joseph’s Healthcare, Hamilton, ON, Canada, ⁷Guy’s Severe Asthma Centre, Guy’s and St Thomas’ NHS Trust; School of Immunology and Microbial Sciences, King’s College London, London, United Kingdom, ⁸Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan, ⁹Late-Stage Respiratory and Immunology, BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ¹⁰BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ¹¹Late-Stage Respiratory and Immunology, BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ¹²National Jewish Health, Denver, CO
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterized by asthma, eosinophilia, and small-to-medium size vessel vasculitis, with individual manifestations widely ranging…
Abstract Number: 1599 • ACR Convergence 2024
Glucocorticoids versus Glucocorticoids Plus Cyclophosphamide in Eosinophilic Granulomatosis with Polyangiitis with Poor-Prognosis Factors: A Target Trial Emulation Study
Boris Sorin¹, Matthias Papo¹, Renato Alberto Sinico², Vítor Silvestre Teixeira³, Nils Venhoff⁴, Maria-Letizia Urban⁵, Michele Iudici⁶, Juliane Mahrhold⁷, Francesco Locatelli⁸, Giulia Cassone⁹, Franco Schiavon¹⁰, Benjamin Seeliger¹¹, Thomas Neumann¹², Claudia Feder¹³, Claus Kroegel¹⁴, Matthieu Groh¹⁵, Chiara Marvisi¹⁶, Maxime Samson¹⁷, Thomas Barba¹⁸, David Jayne¹⁹, Arianna Troilo²⁰, Jens Thiel²⁰, Bernhard Hellmich²¹, Sara Monti²², Carlomaurizio Montecucco²³, Carlo Salvarani²⁴, Jean-Emmanuel Kahn²⁵, Bernard Bonnotte²⁶, Cécile-Audrey Durel²⁷, Xavier Puéchal²⁸, Luc Mouthon²⁹, Loïc Guillevin³⁰, Giacomo Emmi³¹, Augusto Vaglio³², Raphael Porcher³³ and Benjamin Terrier³⁴, and the French Vasculitis Study Group (FVSG) and the European EGPA Study Group (EESG), ¹Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris, Paris, France, ²Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Milan, Italy, ³Department of Rheumatology, Faro Hospital, Algarve, Portugal, ⁴Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris and Division of rheumatology, Geneva University Hospitals and University of Geneva, Geneva, Switzerland, Paris, France, ⁷Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabtologyy and Immunology, ERN-RITA Reference Center, Vasculitis-Center Tübingen-Kirchheim, Medius Kliniken Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, ⁸Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy, ⁹Clinical and Experimental Medicine PhD Program, Azienda USL-IRCCS di Reggio Emilia and Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁰Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ¹¹Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany, ¹²Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany and Division of Rheumatology, Immunology and Rehabilitation, Kantonsspital St Gallen, St Gallen, Switzerland, Jena, Germany, ¹³Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany, ¹⁴Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany, ¹⁵Foch, Suresnes, France, ¹⁶Rheumatology Unit Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁷Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France, ¹⁸Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, ¹⁹University of Cambridge, Cambridge, United Kingdom, ²⁰Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, Freiburg, Germany, ²¹Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, ²²IRCCS Istituto Auxologico Italiano, Milan, Italy, ²³IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ²⁴Azienda USL-IRCCS di Reggio Emilia and University of Modena and Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy, ²⁵Department of Internal Medicine, Ambroise Paré Hospital, Assistance Publique - Hôpitaux de Paris, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France, Boulogne Billancourt, France, ²⁶Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France., Dijon, France, ²⁷Edouard Herriot University Hospital, Lyon, France, ²⁸National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ²⁹Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l'Est et de l'Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ³⁰National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ³¹Department of Medical, Surgery and Health Sciences, University of Trieste, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, ³²Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Florence, Italy, ³³Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, ³⁴Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis characterized by asthma, blood and tissue eosinophilia and vasculitis affecting multiple organs. According to…
Abstract Number: 1600 • ACR Convergence 2024
Glucocorticoids versus Glucocorticoids Plus Cyclophosphamide in Eosinophilic Granulomatosis with Polyangiitis Without Poor-Prognosis Factors: A Target Trial Emulation Study
Boris Sorin¹, Matthias Papo¹, Renato Alberto Sinico², Vítor Silvestre Teixeira³, Nils Venhoff⁴, Maria-Letizia Urban⁵, Michele Iudici⁶, Juliane Mahrhold⁷, Francesco Locatelli⁸, Giulia Cassone⁹, Franco Schiavon¹⁰, Benjamin Seeliger¹¹, Thomas Neumann¹², Claudia Feder¹³, Claus Kroegel¹⁴, Matthieu Groh¹⁵, Chiara Marvisi¹⁶, Maxime Samson¹⁷, Thomas Barba¹⁸, David Jayne¹⁹, Arianna Troilo²⁰, Jens Thiel²⁰, Bernhard Hellmich²¹, Sara Monti²², Carlomaurizio Montecucco²³, Carlo Salvarani²⁴, Jean-Emmanuel Kahn²⁵, Bernard Bonnotte²⁶, Cécile-Audrey Durel²⁷, Xavier Puéchal²⁸, Luc Mouthon²⁹, Loïc Guillevin³⁰, Giacomo Emmi³¹, Augusto Vaglio³², Raphael Porcher³³ and Benjamin Terrier³⁴, and the French Vasculitis Study Group (FVSG) and the European EGPA Study Group (EESG), ¹Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris, Paris, France, ²Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Milan, Italy, ³Department of Rheumatology, Faro Hospital, Algarve, Portugal, ⁴Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris and Division of rheumatology, Geneva University Hospitals and University of Geneva, Geneva, Switzerland, Paris, France, ⁷Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabtologyy and Immunology, ERN-RITA Reference Center, Vasculitis-Center Tübingen-Kirchheim, Medius Kliniken Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, ⁸Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy, ⁹Clinical and Experimental Medicine PhD Program, Azienda USL-IRCCS di Reggio Emilia and Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁰Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ¹¹Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany, ¹²Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany and Division of Rheumatology, Immunology and Rehabilitation, Kantonsspital St Gallen, St Gallen, Switzerland, Jena, Germany, ¹³Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany, ¹⁴Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany, ¹⁵Foch, Suresnes, France, ¹⁶Rheumatology Unit Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁷Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France, ¹⁸Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, ¹⁹University of Cambridge, Cambridge, United Kingdom, ²⁰Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, Freiburg, Germany, ²¹Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, ²²IRCCS Istituto Auxologico Italiano, Milan, Italy, ²³IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ²⁴Azienda USL-IRCCS di Reggio Emilia and University of Modena and Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy, ²⁵Department of Internal Medicine, Ambroise Paré Hospital, Assistance Publique - Hôpitaux de Paris, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France, Boulogne Billancourt, France, ²⁶Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France., Dijon, France, ²⁷Edouard Herriot University Hospital, Lyon, France, ²⁸National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ²⁹Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l'Est et de l'Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ³⁰National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ³¹Department of Medical, Surgery and Health Sciences, University of Trieste, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, ³²Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Florence, Italy, ³³Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, ³⁴Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis characterized by asthma, blood and tissue eosinophilia and vasculitis affecting multiple organs. According to the…
Abstract Number: 1601 • ACR Convergence 2024
Changing Spectrum of Systemic Therapies for Eosinophilic Granulomatosis with Polyangiitis from 2006-2023
Lynn Fussner¹, Christian Pagnoux², David Cuthbertson³, Thomas Corbridge⁴, Nader Khalidi⁵, Curry Koening⁶, Carol Langford⁷, Carol McAlear⁸, Paul Monach⁹, Larry Moreland¹⁰, Rennie Rhee⁸, Philip Seo¹¹, Anat Shavit¹², Jared Silver¹³, Ulrich Specks¹⁴, Kenneth Warrington¹⁴, Michael Wechsler¹⁵ and Peter Merkel⁸, and for the Vasculitis Clinical Research Consortium, ¹The Ohio State University, Columbus, OH, ²Mount Sinai Hospital, Toronto, ON, Canada, ³University of South Florida, Tampa, FL, ⁴US Value Evidence and Outcomes, GSK, Durham, NC, ⁵McMaster University, Hamilton, ON, Canada, ⁶University of Texas Dell Medical School, Austin, TX, ⁷Cleveland Clinic, Moreland Hills, OH, ⁸University of Pennsylvania, Philadelphia, PA, ⁹VA Boston Healthcare System, Boston, MA, ¹⁰University of Colorado, Denver, CO, ¹¹Johns Hopkins University, Baltimore, MD, ¹²BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ¹³US Medical Affairs - Respiratory, GSK, Durham, ¹⁴Mayo Clinic, Rochester, MN, ¹⁵National Jewish Health, Denver, CO
Background/Purpose: The emergence of biologic therapies targeting eosinophils via the IL-5 pathway provided new options for treatment in eosinophilic granulomatosis with polyangiitis (EGPA). There has…
Abstract Number: 1604 • ACR Convergence 2024
Utilizing Machine Learning with Claims Data to Diagnose and Quantify the Prevalence of Eosinophilic Granulomatosis with Polyangiitis
Peter Merkel¹, Peter Baudy², Donna Carstens², Benjamin North³, Mahvish Danka³, Stephanie Roy³, Hanna Marshall³ and Geoffrey Chupp⁴, ¹University of Pennsylvania, Philadelphia, PA, ²Respiratory & Immunology US Medical, AstraZeneca, Wilmington, ³IQVIA, Philadelphia, ⁴School of Medicine, Yale University, New Haven
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis that can present indolently. This can lead to a delay in diagnosis and treatment. This study…
Abstract Number: 1605 • ACR Convergence 2024
Characteristics of Relapses in Patients with Eosinophilic Granulomatosis with Polyangiitis
Peter Merkel¹, David Jayne², Ulrich Specks³, Christian Pagnoux⁴, Parameswaran Nair⁵, Nader Khalidi⁵, Arnaud Bourdin⁶, Lena Börjesson Sjö⁷, Sofia Necander⁷, Anat Shavit⁸, Claire Walton⁹ and Michael Wechsler¹⁰, ¹University of Pennsylvania, Philadelphia, PA, ²University of Cambridge, Cambridge, United Kingdom, ³Mayo Clinic, Rochester, MN, ⁴Mount Sinai Hospital, Toronto, ON, Canada, ⁵McMaster University and St Joseph’s Healthcare, Hamilton, ON, Canada, ⁶Department of Respiratory Diseases, University of Montpellier, CHU Montpellier, PhyMedExp, INSERM, CNRS, Montpellier, France, ⁷Late-Stage Respiratory and Immunology, BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁸BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ⁹Late-Stage Respiratory and Immunology, BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ¹⁰National Jewish Health, Denver, CO
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterized by asthma, eosinophilia, and small-to-medium size vessel vasculitis. In the Phase 3, double-blind,…
Abstract Number: 1606 • ACR Convergence 2024
Demographic and Treatment Patterns in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Cohort Analysis of US Claims and Clinical Data
Paul Dolin¹, Anat Shavit¹, Karina A. Keogh², Jennifer Rowell³, Chris Edmonds⁴, James M. Eudicone⁵, Amanda M. Moore⁶, Melissa Wilson⁷, Kinwei A. Chan⁶, Thomas Peer⁸ and Stephanie Y. Chen⁹, ¹BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ²Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Mayo Clinic,, Rochester, MN, ³Market Access and Pricing, AstraZeneca, Cambridge, England, United Kingdom, ⁴Market Access and Pricing, AstraZeneca, Gaithersburg, MD, ⁵Real World Science and Analytics, BioPharmaceuticals Medical, AstraZeneca, Wilmington, DE, ⁶TriNetX, Cambridge, MA, ⁷TriNetX, Durham, NC, ⁸TriNetX, Farmington, NY, ⁹BioPharmaceuticals Medical, AstraZeneca, Gaithersburg, MD
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterized by chronic necrotizing vasculitis. Conventional therapy (oral glucocorticoids [OGC] ± immunosuppressants), mepolizumab, and…
Abstract Number: 1607 • ACR Convergence 2024
Low Concentrations of Anti-C5a Complement Receptor Antibodies Are Associated with Relapse in Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Sebastian Klapa¹, Sabrina Arnold², Antje Müller², Andreas Koch³, Anja Staehle⁴, Wataru Kähler³, Harry Heidecke⁵, Gabriela Riemekasten² and Peter Lamprecht², ¹University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, ²University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, ³Christian-Albrechts-University Kiel, Institute of Experimental Medicine, Kiel, Germany, ⁴University of Lübeck, Department of Rheumatology and Immunology, Lübeck, Germany, ⁵CellTrend GmbH, Luckenwalde, Germany
Background/Purpose: Complement activation has been shown to play an important role in the pathogenesis of the two major anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) variants,…
Abstract Number: 1608 • ACR Convergence 2024
Interstitial Lung Disease in ANCA-associated Vasculitis: A Retrospective Study of Clinical Characteristics, Radiographic Features, and Outcomes
Janelle Castellino¹, Matas Orentas², Joshua Moran³, Joshlean Fair¹, Yanyu Zhang⁴ and Aleksandra Bukiej⁵, ¹Rush University Medical Center, Chicago, IL, ²RUMC, Chicago, IL, ³Rush University Medical Center - IM Residency, Chicago, IL, ⁴Rush University Medical Center, Chicago, ⁵Rush University Medical Center, River Forest, IL
Background/Purpose: Interstitial lung disease (ILD) is a significant manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and…
Abstract Number: 1611 • ACR Convergence 2024
Impact of Mepolizumab on Patient-reported Outcomes in Eosinophilic Granulomatosis with Polyangiitis by Using the ANCA-associated Vasculitis Patient-reported Outcomes (AAV-PRO) Questionnaire: A European Multicentre Prospective Study
Paolo Delvino¹, Luca Quartuccio², Joanna Robson³, Federico Alberici⁴, Diego Bagnasco⁵, Alvise Berti⁶, Marco Caminati⁷, Marta Camoni⁸, Maria Cid⁹, Edoardo Conticini¹⁰, Giulia Costanzo¹¹, Claire de Moreuil¹², Stefano Del Giacco¹¹, Georgina Espigol-Frigole¹³, Virginia V Ferretti¹⁴, Franco Franceschini¹⁵, Luca Iorio¹⁶, Anna Kernder¹⁷, Catherine Klersy¹⁴, Alberto Lo Gullo¹⁸, Laura Losappio¹⁹, Elena Manna²⁰, Matteo Maule²¹, Carlomaurizio Montecucco²², Negrini Simone²³, Roberto Padoan²⁴, Francesca Regola²⁵, Luisa Ricciardi²⁶, Jan Schroeder¹⁹, Benjamin Terrier²⁷, Paola Toniati²⁸, Elena Treppo², Maria-Letizia Urban²⁹, Augusto Vaglio³⁰, Giacomo Emmi³¹ and Sara Monti³², and European EGPA Study Group, ¹University of Milano-Bicocca, Milan, Milan, Italy, ²Division of Rheumatology, Department of Medicine (DMED), University of Udine, Udine, Italy, Udine, Italy, ³UWE Bristol, Bristol, United Kingdom, ⁴Nephrology Unit, University of Brescia, ASST Spedali Civili, University of Brescia, Brescia, Italy, Brescia, Italy, ⁵Allergy and Respiratory Diseases, Department of Internal Medicine (DIMI), IRCCS Policlinico San Martino, Genoa, Italy, ⁶University of Trento, Rochester, Italy, ⁷Department of Medicine, Asthma, Allergy and Clinical Immunology Section, University of Verona, Verona, Italy, ⁸Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Brescia, Italy, ⁹Hospital Clinic Barcelona. University of Barcelona, Barcelona, Spain, ¹⁰University of Siena, Siena, Siena, Italy, ¹¹Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy, ¹²Service de médecine interne, CHU de Brest, Brest, France, ¹³Hospital Clinic de Barcelona, Barcelona, Spain, ¹⁴Biostatistcs & Clinical Trial Center, Fondazione IRCCS Policlinico San Matteo, Pavia, Pavia, Italy, ¹⁵Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ¹⁶Rheumatology Unit, Department of Medicine DIMED, University of Padua, Padova, Italy, Padova, Italy, ¹⁷HHU Dusseldorf, Dusseldorf, Germany, ¹⁸Rheumatology Unit, Papardo Hospital, Messina, Italy, ¹⁹Division of Allergy and Clinical Immunology, ASST GOM Niguarda, Milan, Milan, Italy, ²⁰Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Pavia, Italy, ²¹Asthma Center and Allergy Unit, Verona Integrated University Hospital, Verona, Verona, Italy, ²²IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ²³Department of Internal Medicine (DIMI), University of Genoa, Genoa, Genoa, Italy, ²⁴Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ²⁵Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili Brescia and University of Brescia, Brescia, Italy, ²⁶Allergy and Immunology Unit, Department of Clinical and Experimental Medicine, G. Martino Teaching Hospital, University of Messina, Messina, Messina, Italy, ²⁷Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, ²⁸Spedali civili, 25123, Italy, ²⁹Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ³⁰Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Florence, Italy, ³¹University of Trieste, Trieste, Italy, ³²IRCCS Istituto Auxologico Italiano, Milan, Italy
Background/Purpose: Mepolizumab (MEPO) proved its efficacy in the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) in the randomized controlled MIRRA trial. The ANCA-associated vasculitis patient-reported…

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