Abstracts tagged "Churg-Strauss syndrome"

Abstract Number: 869 • 2019 ACR/ARP Annual Meeting
Clinical Manifestations of Patients with Eosinophilic Granulomatosis with Polyangiitis in a Large North American Cohort
Irena Doubelt¹, David Cuthbertson ², Simon Carette ¹, Nader A. Khalidi ³, Curry L. Koening ⁴, Carol Langford ⁵, Carol A. McAlear ⁶, Larry W. Moreland ⁷, Paul Monach ⁸, Philip Seo ⁹, Ulrich Specks ¹⁰, Antoine Sreih ¹¹, Steven Ytterberg ¹⁰, Peter A. Merkel ¹², Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹³, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²University of South Florida, Tampa, FL, ³McMaster University, Hamilton, ON, Canada, ⁴University of Utah Hospital, Salt Lake City, UT, ⁵Cleveland Clinic, Cleveland, OH, ⁶University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸Brigham and Women's Hospital, Boston, MA, ⁹Johns Hopkins Medicine, Baltimore, MD, ¹⁰Mayo Clinic College of Medicine, Rochester, MN, ¹¹University of Pennsylvania, Philadelphia, PA, ¹²Univeristy of Pennsylvania, Philadelphia, PA, ¹³University of Pennsylvania, Division of Rheumatology, philadelphia
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing small-vessel vasculitis, with only a few published North American series. This project aimed to…
Abstract Number: 870 • 2019 ACR/ARP Annual Meeting
Off-Label Use of Biotherapies to Treat Relapsing And/or Refractory Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)
Alice Canzian ¹, Nils Venhoff ², Silvia Sartorelli ³, Anne-Marie Ruppert ⁴, Matthieu Groh ⁵, Camille Taille ⁶, Virginie Rieu ⁷, Perrine Smets ⁷, François Maurier ⁸, Nicolas Girszyn ⁹, Maxime Samson ¹⁰, Claire de Moreuil ¹¹, Grégory Pugnet ¹², Xavier Delbrel ¹³, Jean-Emmanuel Kahn ¹⁴, Xavier Puéchal for the French Vasculitis Study Group ¹⁵, Giacomo Emmi ¹⁶, Loic Guillevin ¹⁵, Lorenzo Dagna ¹⁷, Jens Thiel ², Augusto Vaglio ¹⁸ and Benjamin Terrier¹⁵, ¹Cochin Hospital, Paris, France, ²Clinic for Rheumatology and Clinical Immunology, Faculty of Medicine, Medical Center, University of Freiburg, Freiburg, Germany, ³Vita-Salute San Raffaele University, IRCCS San Raffaele Hospital, Milan, Italy, ⁴Tenon Hospital, Paris, France, ⁵Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, Suresnes, France, ⁶Bichat Hospital, Paris, France, ⁷CHU, Clermont-Ferrand, France, ⁸Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, ⁹CHU, Rouen, France, ¹⁰Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, ¹¹CHU Brest, Brest, France, ¹²CHU de Toulouse, Hôpital Purpan, Service de Médecine Interne, Toulouse, France, ¹³CH, Pau, France, ¹⁴APHP, Boulogne Billancourt, France, ¹⁵National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, ¹⁶Department of Experimental and Clinical Medicine, University of Firenze, Florence, Italy, Florence, Italy, ¹⁷Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy, ¹⁸Nephrology Unit, Parma University Hospital, Parma, Italy, Nephrology Unit, Parma University Hospital, Parma, Italy, Italy
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg–Strauss syndrome), is characterized by small-vessel necrotizing vasculitis, and blood and tissue eosinophilia in asthmatic individuals. Glucocorticoids (GCs)…
Abstract Number: 1683 • 2019 ACR/ARP Annual Meeting
Cardiac Involvement of Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss): Initial Manifestations and Outcomes Based on Data from a Monocenter Patient Cohort
Silvia Sartorelli ¹, Pascal Cohen ², Bertrand Dunogue ², Alexis Régent ², Xavier Puéchal for the French Vasculitis Study Group ², Luc Mouthon ², Loic Guillevin ² and Benjamin Terrier², ¹Vita-Salute San Raffaele University, IRCCS San Raffaele Hospital, Milan, Italy, ²National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by blood and tissue eosinophilia, and asthma. Its cardiac involvement is a major…
Abstract Number: 2619 • 2019 ACR/ARP Annual Meeting
Rituximab for Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review of Observational Studies
Giovanni Pomponio¹, Vincenzo Menditto ², Giulia Rossetti ³, Alessia Angeletti ³, Diletta Olivari ³ and Armando Gabrielli ³, ¹Clinica Medica Ospedali Riuniti di Ancona, Ancona, Italy, ²Internal Medicina Ospedali Riuniti di Ancona, ancona, Italy, ³Clinica Medica Università Politecnica delle Marche, Ancona, Italy
Background/Purpose: More than 40 studies containing data about adults affected by EGPA treated with Rituximab (RTX) have been published in the last decade. Nevertheless, due…
Abstract Number: 2631 • 2019 ACR/ARP Annual Meeting
Increased Risk of Acute Coronary Artery Disease and Ischemic Stroke in Patients with Eosinophilic Granulomatosis with Polyangiitis
Morgane Mourguet ¹, Dominique Chauveau ², Stan Faguer ², JB Ruidavets ², Yannick Béjot ³, Grégoire Prevot ⁴, Olivier Lairez ², David Ribes ², Antoine Huart ², Laurent Alric ², Leo Astudillo ¹, Laurent Sailler ¹ and Grégory Pugnet⁵, ¹CHU Purpan, Toulouse, France, ²CHU Rangueil, Toulouse, France, ³CHU Dijon, Dijon, France, ⁴CHU Larrey, Toulouse, France, ⁵CHU de Toulouse, Hôpital Purpan, Service de Médecine Interne, Toulouse, France
Background/Purpose: Current treatment approaches have greatly improved the survival of eosinophilic granulomatosis with Polyangiitis (EGPA) patients, however both morbidity andmortality are still significant mainly due…
Abstract Number: 2638 • 2019 ACR/ARP Annual Meeting
The Cumulative Burden of Damage for Patients with Eosinophilic Granulomatosis with Polyangiitis
Irena Doubelt¹, David Cuthbertson ², Gunnar Tomasson ³, Simon Carette ¹, Nader A. Khalidi ⁴, Curry L. Koening ⁵, Carol Langford ⁶, Carol A. McAlear ⁷, Larry W. Moreland ⁸, Paul Monach ⁹, Philip Seo ¹⁰, Ulrich Specks ¹¹, Antoine Sreih ¹², Kalen Young ¹³, Steven Ytterberg ¹¹, Peter A. Merkel ¹⁴, Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹⁵, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²University of South Florida, Tampa, FL, ³Faculty of Medicine, University of Iceland and Landpitali University Hospital, Reykjavik, Iceland, ⁴McMaster University, Hamilton, ON, Canada, ⁵University of Utah Hospital, Salt Lake City, UT, ⁶Cleveland Clinic, Cleveland, OH, ⁷University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ⁸University of Pittsburgh, Pittsburgh, PA, ⁹Brigham and Women's Hospital, Boston, MA, ¹⁰Johns Hopkins Medicine, Baltimore, MD, ¹¹Mayo Clinic College of Medicine, Rochester, MN, ¹²University of Pennsylvania, Philadelphia, PA, ¹³Vasculitis Foundation, Kansas City, MO, ¹⁴Univeristy of Pennsylvania, Philadelphia, PA, ¹⁵University of Pennsylvania, Division of Rheumatology, philadelphia
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by asthma and other manifestations of vasculitis, some of which can be life-threatening, cause major organ damage,…
Abstract Number: 2639 • 2019 ACR/ARP Annual Meeting
Comparison of Patient Self-reported Data to Physician-driven Cohorts in Patients with Eosinophilic Granulomatosis with Polyangiitis
Irena Doubelt¹, Jason Springer ², Tanaz Kermani ³, Antoine Sreih ⁴, Cristina Burroughs ⁵, David Cuthbertson ⁵, Simon Carette ¹, Nader A. Khalidi ⁶, Curry L. Koening ⁷, Carol Langford ⁸, Carol A. McAlear ⁹, Larry W. Moreland ¹⁰, Paul Monach ¹¹, Dianne Shaw ¹², Philip Seo ¹³, Ulrich Specks ¹⁴, Kalen Young ¹⁵, Steven Ytterberg ¹⁴, Peter A. Merkel ¹⁶, Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹⁷, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²Kansas University Medical Center, Kansas, MO, ³University of California Los Angeles, Los Angeles, CA, ⁴University of Pennsylvania, Philadelphia, PA, ⁵University of South Florida, Tampa, FL, ⁶McMaster University, Hamilton, ON, Canada, ⁷University of Utah Hospital, Salt Lake City, UT, ⁸Cleveland Clinic, Cleveland, OH, ⁹University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ¹⁰University of Pittsburgh, Pittsburgh, PA, ¹¹Brigham and Women's Hospital, Boston, MA, ¹²Vasculitis Foundation, North Carolina, ¹³Johns Hopkins Medicine, Baltimore, MD, ¹⁴Mayo Clinic College of Medicine, Rochester, MN, ¹⁵Vasculitis Foundation, Kansas City, MO, ¹⁶Univeristy of Pennsylvania, Philadelphia, PA, ¹⁷University of Pennsylvania, Division of Rheumatology, philadelphia
Background/Purpose: Aligning perspectives of patients and physicians in the diagnosis and management of diseases is imperative, particularly in rare, chronic diseases such as vasculitis. We…
Abstract Number: 908 • 2018 ACR/ARHP Annual Meeting
Early Prediction of Long-Term Evolutionary Profiles of Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss) Based on Baseline and Follow-up Characteristics
Matthias Papo¹, Giacomo Emmi², Franco Schiavon³, Matthieu Groh⁴, Maria-Letizia Urban², Chiara Marvisi⁵, Jean-Emmanuel Kahn⁴, Alberto Sinico⁶, Maxime Samson⁷, Pascal Cohen¹, Xavier Puéchal¹, Luc Mouthon¹, Loïc Guillevin¹, Augusto Vaglio⁵ and Benjamin Terrier⁸, ¹Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, ²Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ³Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy, ⁴Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, ⁵Nephrology Unit, Parma University Hospital, Parma, Italy, ⁶UOS di Immunologia Clinica e UOC di Nefrologia, Azienda Ospedaliera San Carlo Borromeo, Milan, Italy, ⁷Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France, ⁸Department of Internal Medicine, Centre de Référence pour les Maladies Auto-immunes Rares, Paris, France, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is a small-vessel necrotizing vasculitis characterized by blood and tissue eosinophilia and asthma. Glucocorticoids (GCs) effectively control the…
Abstract Number: 1752 • 2018 ACR/ARHP Annual Meeting
Asthma in Eosinophilic Granulomatosis with Polyangiitis Treated with Rituximab
Marta Casal Moura¹, Alvise Berti², Karina Keogh², Gerald Volcheck³, Ulrich Specks⁴ and Misbah Baqir⁵, ¹Pulmonary and Critical Care, Thoracic Disease Research Unit, Mayo Clinic College of Medicine, Rochester, MN, ²Pulmonary and Critical Care, Mayo Clinic College of Medicine, Rochester, MN, ³Allergic Diseases, Internal Medicine Department, Mayo Clinic College of Medicine, Rochester, MN, ⁴Mayo Clinic College of Medicine, Rochester, MN, ⁵Pulmonary/Critical Care, Mayo Clinic College of Medicine, Rochester, MN
Background/Purpose: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic small-vessel necrotizing vasculitis presenting with asthma and eosinophilia. Anti-neutrophil cytoplasmic antibodies (ANCA) association may influence…
Abstract Number: 1754 • 2018 ACR/ARHP Annual Meeting
Clinical and Economic Characteristics of Patients Diagnosed with Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome) in the United States
Christopher F Bell¹, Matthew Lau¹ and Qin Shen², ¹GlaxoSmithKline, Research Triangle Park, NC, ²GlaxoSmithKline, Collegeville, PA
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss Syndrome, is a rare, complex multisystem disorder belonging to a group of autoimmune inflammatory diseases characterized by…
Abstract Number: 1755 • 2018 ACR/ARHP Annual Meeting
Burden of Illness Associated with Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome): Evidence from a Managed Care Database in the United States
Christopher F Bell¹, Cori Blauer-Peterson² and Jianbin Mao², ¹GlaxoSmithKline, Research Triangle Park, NC, ²Optum, Eden Prairie, MN
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA), is a rare, complex multisystem disorder, characterized by vascular inflammation and multisystem organ damage. EGPA manifests as asthma, rhinosinusitis,…
Abstract Number: 1756 • 2018 ACR/ARHP Annual Meeting
Systematic Review of the Clinical Effectiveness of Treatments in Eosinophilic Granulomatosis with Polyangiitis
Scott Doyle¹, Annete Njue², Matthew Lyall², Rebecca Rushton², Anne Heyes² and Maebh Kelly³, ¹Value Evidence and Outcomes, GlaxoSmithKline, London, United Kingdom, ²RTI Health Solutions, Manchester, United Kingdom, ³Pope Woodhead, London, United Kingdom
Background/Purpose: To better understand the available clinical effectiveness data in eosinophilic granulomatosis with polyangiitis (EGPA), a systematic literature review was undertaken. The primary objective of…
Abstract Number: 1757 • 2018 ACR/ARHP Annual Meeting
An Economic Systematic Literature Review of Eosinophilic Granulomatosis with Polyangiitis
Scott Doyle¹, Emily Moss², Louise Hartley², Chris Knight², Judith Bell², Outi Ahdesmäki² and Maebh Kelly³, ¹Value Evidence and Outcomes, GlaxoSmithKline, London, United Kingdom, ²RTI Health Solutions, Manchester, United Kingdom, ³Pope Woodhead, London, United Kingdom
Background/Purpose: To better understand the available economic evaluations in eosinophilic granulomatosis with polyangiitis (EGPA), a systematic literature review was undertaken. The primary objective of this…
Abstract Number: 1758 • 2018 ACR/ARHP Annual Meeting
A 24 Month Analysis of Rituximab Safety and Efficacy in Eosinophilic Granulomatosis with Polyangiitis
Vítor Teixeira^1,2, Aladdin Mohammad^1,3 and David Jayne¹, ¹Department of Medicine, University of Cambridge, Cambridge, United Kingdom, ²Rheumatology Department, CHLN - Santa Maria Hospital, Lisbon Academic Medical Center, Lisbon, Portugal, ³Rheumatology, Department of Clinical Sciences, Lund, Lund University, Lund, Sweden
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) frequently pursues a refractory course leading to high glucocorticoid exposure and toxicity. A previous retrospective study found rituximab (RTX)…
Abstract Number: 1774 • 2017 ACR/ARHP Annual Meeting
Serum Periostin As a Biomarker in Eosinophilic Granulomatosis with Polyangiitis
Rennie L. Rhee¹, Cecile TJ Holweg², David Cuthbertson³, Simon Carette⁴, Nader A. Khalidi⁵, Curry L. Koening⁶, Jeffrey Krischer⁷, Carol A. Langford⁸, Carol A. McAlear⁹, Paul A. Monach¹⁰, Larry W. Moreland¹¹, Christian Pagnoux⁴, Philip Seo¹², Ulrich Specks¹³, Antoine G. Sreih¹, Steven R. Ytterberg¹⁴ and Peter A. Merkel¹⁵, ¹Rheumatology, University of Pennsylvania, Philadelphia, PA, ²Genentech, Inc, South San Francisco, CA, ³Biostatistics and Informatics, Department of Pediatrics, University of South Florida, Tampa, FL, ⁴Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Rheumatology, McMaster University, Hamilton, ON, Canada, ⁶Rheumatology, University of Utah, Salt Lake City, UT, ⁷University of South Florida, Tampa, FL, ⁸Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, ⁹University of Pennsylvania, Philadelphia, PA, ¹⁰Boston University School of Medicine, Boston, MA, ¹¹Division of Rheumatology and Clinical Immunology, UPMC / University of Pittsburgh, Pittsburgh, PA, ¹²Medicine, Johns Hopkins University, Baltimore, MD, ¹³Mayo Clinic College of Medicine, Rochester, MN, ¹⁴Rheumatology, Mayo Clinic, Rochester, MN, ¹⁵Division of Rheumatology, University of Pennsylvania, Philadelphia, PA
Background/Purpose: Identification of a biomarker to predict relapse in eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss) would enhance the ability to personalize treatment options and improve…

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