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

  • Abstract Number: 2532 • ACR Convergence 2025

    Temporal arteritis revealing eosinophilic diseases: A multicenter retrospective study, literature review, and combined cluster analysis

    Julie Merindol1, Benjamin Torreau2, Matthieu Groh3, Amy Klion4, Paolo Delvino5, Olivier Espitia6, Jean-Benoit Arlet7, Sarah Lechtman8, Jean-sébastien Allain9, Federica Bello10, Anaelle Boucaud11, Florian Catros12, Julien Campagne13, Anne Coutard14, Cecile-Audrey DUREL15, Mikael Ebbo16, Georgina Espigol-Frigole17, Giacomo Emmi18, Yanis Kouchit19, Guillaume Lefevre20, François Lifermann21, PAUL LEGENDRE22, Eric Liozon23, Francois Maurier24, Sara Melboucy Belhkir25, Sébastien Miranda26, Simon Parreau23, Paola Parronchi27, Jacques Pouchot28, Vincent Pestre29, Sophie Rosenstingl30, Jan Willem Cohen Tervaert31, Odile Souchaud Debouverie32, Xavier Puéchal33 and Benjamin Terrier34, 1CHU de Nice, Nice, France, 2Internal Medicine and Immunology, CHU Tours, Tours, France, 3Hôpital Foch, paris, France, 4NIAID/NIH, Bethesda, MD, 5University of Milano-Bicocca, Monza, Monza and Brianza, Italy, 6CHU de Nantes, Nantes, France, 7APHP, Paris, 8CHU de Nice, Nixe, France, 9Scorff-Lorient Hospital, Lorient, France, 10Université de Florence, Florence, Italy, 11CHU de Tours, Tours, France, 12CHI VAL, Ariège, France, 13Hopital Robert Schuman, Metz, France, 14Groupe Hospitalier de Bretagne Sud, Lorient, France, 15CHU de Lyon, Lyon, France, 16Internal Medicine, Marseille-APHM, hôpital La Timone, Marseille, France, 17Hospital Clínic de Barcelona, Barcelona, Spain, 18Department of Medical, Surgery and Health Sciences, University of Trieste, Italy and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, Trieste, Italy, 19CH de Cannes, Cannes, France, 20CHU de Lille, Lille, France, 21Internal Medicine, CH de Dax, Dax, France, 22CH LE MANS, Le Mans, France, 23CHU de Limoges, Limoges, France, 24HOPITAUX PRIVES DE METZ, Vaux / Frankreich, France, 25CH de Saint Quentin, Saint Quentin, France, 26Internal Medicine, CHU Rouen, Rouen, France, 27CHU de Florence, Florence, Italy, 28AP-HP, Rueil Malmaison, France, 29CH d'Avignon, Avignon, France, 30CH de Compiegne, Compiegne, France, 31University of Alberta, Edmonton, AB, Canada, 32CHU de Poitiers, Poitiers, France, 33Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, and Université Paris Cité, Paris ( 75014 ), Ile-de-France, France, 34Cochin Hospital, Paris, France

    Background/Purpose: Temporal arteritis (TA) classically presents as a large-vessel vasculitis in older adults with cranial ischemic symptoms. However, in younger patients or in the presence…
  • Abstract Number: 2514 • ACR Convergence 2025

    Comparison of Demographics and Outcomes of Inpatient Hospitalization of Severe Asthma Patients with Eosinophilic Granulomatosis with Polyangitis. A Study on the National Inpatient Sample Database

    Prakriti Subedi1, Anil Regmi2, Karun Shrestha3, Manoj Ghimire4, Sajana Poudel5, Kalpana Ghimire1, Oshna Pandey1 and Clement Tagoe6, 1St. Barnabas Hospital, Bronx, NY, 2Parkview Health, Fort Wayne, IN, 3St. Barnabas Hospital Internal Medicine Residency, Bronx, NY, 4St Barnabas Hospital, BRONX, NY, 5Cook County Health, CHICAGO, NY, 6Albert Einstein College of Medicine, FRESH MEADOWS, NY

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis marked by asthma and eosinophilia, often leading to severe, persistent respiratory symptoms. Unlike severe asthma…
  • 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…
  • Abstract Number: 0827 • ACR Convergence 2024

    Oral Corticosteroid-Sparing Effects of Mepolizumab in Eosinophilic Granulomatosis with Polyangiitis (EGPA): Results up to 7.4 Years from the Long-Term Access Programme

    Paneez Khoury1, Jared Silver2, Gerhard Wolff3, Robert Price4, Rejina Verghis5, Emmeline Igboekwe2 and Michael E Wechsler6, 1National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 2US Medical Affairs-Respiratory, GSK, Durham, 3Clinical Development-Respiratory, GSK, Collegeville, 4Biostatistics, GSK, Stevenage, United Kingdom, 5Biostatistics, Development Respiratory, GSK, Brentford, United Kingdom, 6Department of Medicine, National Jewish Health, Denver, CO

    Background/Purpose: EGPA is a severe, rare, relapsing/remitting inflammatory disease, in which chronic or high oral corticosteroid (OCS) doses lead to adverse effects, adding to disease…
  • Abstract Number: 2000 • ACR Convergence 2024

    Induction of Systemic Inflammatory Diseases with Dupilumab Therapy

    Jeanne Tisseau des Escotais1, Camille Taille2, Julie Merindol3, Matthieu Groh4, Perrine SMETS5, Amel Boudjemaa6, Alexandra Audemard7, Nabil Belfeki8, Philippe Bonniaud9, Chloe Comarmond10, Isabelle DELACROIX11, Charlotte Descours12, Lucile Grange13, Paul Legendre14, Nihal Martis15, Thomas Moulinet16, Béatrice Walls17, Laurent Chouchana1 and Benjamin Terrier18, 1Cochin, Paris, France, 2Bichat Hospital, Paris, France, 3Internal Medicine Department, University Hospital of Nice, Côte d'Azur University, Nice, Provence-Alpes-Côte d'Azur, France., Nice, France, 4Foch, Suresnes, France, 5Clermont Ferrand University Hospital - National reference center for autoimmune disease, Internal Medicine, Clermont-Ferrand, France, 6CHU créteil, Créteil, France, 7Tours, Tours, France, 8CH Melun, Melun, France, 9Centre 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, 10Lariboisière University Hospital, Paris, France, 11CHI créteil, Créteil, France, 12CH Tours, Tours, France, 13CH Saint-Etienne, Saint-Etienne, France, 14Service Médecine Interne et Polyvalente, Centre Hospitaliers Le Mans, Le Mans, France, 15CH Nice, Nice, France, 16CHRU de Nancy, Vandœuvre-lès-Nancy, France, 17Paris, Paris, France, 18Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

    Background/Purpose: Dupilumab is a monoclonal antibody directed against the alpha subunit of the interleukin (IL)-4 receptor, blocking interleukin (IL)-4 and IL-13 signaling. It is approved…
  • Abstract Number: 0828 • ACR Convergence 2024

    Efficacy of Eosinophil-Targeting Therapies on Specific Disease Manifestations of Eosinophilic Granulomatosis with Polyangiitis in the Phase 3 MANDARA Trial

    Peter Merkel1, David Jayne2, Ulrich Specks3, Christian Pagnoux4, Benjamin Terrier5, Bernhard Hellmich6, 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, 5Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, 6Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, 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 small- to medium-vessel vasculitis, asthma and eosinophilia. In the head-to-head MANDARA trial…
  • Abstract Number: 2479 • ACR Convergence 2024

    Mepolizumab Treatment Decreased Oral Corticosteroid Use and Improved Clinical Response, Control Status, and Remission in Patients with Eosinophilic Granulomatosis with Polyangiitis: Results up to 24 Months from a Large Network of US Allergy Practices

    Michael E Wechsler1, Anna Kovalszki2, Jared Silver3, Brian Stone4, Lior Seluk5, Lynn Huynh6, Wilson da Costa Junior6, Mingchen Ye6, Jeremiah Hwee7, Mei Sheng Duh6, William McCann4 and Amy G. Edgecomb8, 1Department of Medicine, National Jewish Health, Denver, CO, 2Division of Allergy and Clinical Immunology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, 3US Medical Affairs - Respiratory, GSK, Durham, 4Allergy Partners, Asheville, NC, 5Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, 6Analysis Group, Boston, MA, 7Value Evidence and Outcomes, GSK, Mississauga, Canada, 8US Value, Evidence, & Outcomes, Anti-Infectives and Respiratory, GSK, Philadelphia

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a severe rare systemic inflammatory disease characterized by elevated blood eosinophil counts ≥1000 cells/µL and vasculitis of small-…
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