ACR Meeting Abstracts

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Abstracts tagged "ANCA associated vasculitis"

  • Abstract Number: 0722 • ACR Convergence 2024

    Factors Associated with Relapses and Performance of the French Vasculitis Study Group Relapse Score in a Cohort of Mexican Patients with ANCA-Associated Vasculitis

    Andrea Hinojosa-Azaola1, Marlon Sandino-Bermúdez2, Ana Sarahí Mulia-Soto3, Juan M. Mejía-Vilet4 and Eduardo Martin Nares5, 1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Mexico City, Mexico, 2Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Mexico City, Distrito Federal, Mexico, 3Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Ciudad de México, Mexico, 4Department of Nephrology and Mineral Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, Mexico City, Mexico, 5Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Background/Purpose: Anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) encompass a group of systemic inflammatory diseases with multi-organ involvement. Relapses occur in 14-44% of patients within 18-36 months…
  • Abstract Number: 0826 • ACR Convergence 2024

    Plasma Exchange for Severe Alveolar Hemorrhage in ANCA-Associated Vasculitis: Emulation of a Target Trial

    Anne-Claire Sanna1, Ségolène Gendreau2, Cyrielle Desnos3, Dimitri Titeca-Beauport4, Jolan Malherbe5, Florian Reizine6, Kladoum Nassarmadji7, Louis Guillaume8, Alban Deroux9, Eddine Bendiab10, Jennifer Catano11, Julien Campagne12, Marc Pineton de Chambrun13, Valentin Coirier14, Clara Vigneron11, Maxime SAMSON15, Antoine Neel16, Benoît Brilland17, Jean-Philippe Martellosio18, Maxim Desgrouas19, Olivier Simon20, Perrine SMETS21, Romain Paule22, Stanislas Faguer23, Stéphane Giorgiutti24, Thylbert Deltombe20, François Arrive18, Ingrid Masson25, Thomas Quemeneur26, Cécile Audrey Durel27, Etienne de Montmollin11, François Perrin28, Herve Lobbes29, Quentin Lajoye30, Rafik Mesbah31, Rodéreau Outh32, Adeline Lacraz33, Damien Contou34, Fabien Parazols35, Florian Garo36, Grégoire Ducoux37, Halil Yildiz38, Isabelle Rey39, Viviane Queyrel40, Rita Eid11, Robin Arcani41, Xavier Puéchal42, Raphael Porcher43, Nicolas de Prost11 and Benjamin Terrier44, and French Vasculitis Study Group (FVSG); Groupe de Recherche en Réanimation Respiratoire du patient d'Onco-Hématologie (GrrrOH), 1Cochin Hospital, Paris, France, 2Mondor Hospital, Paris, 3Tenon Hospital, Paris, France, 4CHU Amiens, Amiens, France, 5CHU Caen, Caen, France, 6CHU Vannes, Vannes, 7APHP, Paris, 8Hopital de Metz, Metz, France, 9Clinique Universitaire de Médecine Interne, Department of Internal Medicine, Grenoble University Hospital, Grenoble, France, 10CHU Montpellier, Montpellier, France, 11APHP, Paris, France, 12Hôpital Robert Schuman - Competence center for autoimmune diseases, Internal Medicine, Metz, France, 13Sorbonne Université, Paris, France, 14CHU Rennes, Rennes, France, 15Dijon University Hospital, Dijon, France, 16Department of Internal Medicine, CHU Nantes, Nantes, France, 17CHU Angers, Angers, France, 18CHU Poitiers, Poitiers, France, 19CHU Orléans, Orleans, France, 20CHU Réunion, Réunion, France, 21Clermont Ferrand University Hospital - National reference center for autoimmune disease, Internal Medicine, Clermont-Ferrand, France, 22Foch Hospital, Suresnes, France, 23CHU Toulouse, Toulouse, France, 24CHRU Strasbourg, Strasbourg, France, 25CHU Saint Etienne, Saint Etienne, France, 26CH Valenciennes, Valenciennes, France, 27HCL, Lyon, France, 28CH Saint Nazaire, Saint Nazaire, France, 29CHU Clermont-Ferrand, Clermont-Ferrand, France, 30CHU Bordeaux, Bordeaux, France, 31CH Boulogne, Boulogne, France, 32CH, Perpignan, France,, Perpignan, France, 33CH Bayonne, Bayonne, France, 34CH Argenteuil, Argenteuil, France, 35CH Perpignan, Perpignan, France, 36CHU Nimes, Nimes, France, 37CHU Lyon, Lyon, France, 38Université de Louvain, Louvain, Belgium, 39CH Annecy, Annecy, France, 40CHU Nice, Nice, France, 41APHM, Marseille, France, 42National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, 43Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, 44Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

    Background/Purpose: Mortality is high in patients with diffuse alveolar hemorrhage (DAH) associated with ANCA-associated vasculitis (AAV). In the PEXIVAS study, plasma exchange (PLEX) did not have a…
  • 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: 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-…
  • Abstract Number: 2497 • ACR Convergence 2024

    Clinical Characteristics and Outcomes of Hydralazine-induced ANCA-associated Vasculitis. a Single Center Retrospective Cohort Analysis from the US

    Muhammad Shamim1, Mazen Allouni2, Haseeb Chaudhary3, Ansaam Daoud4, Loai Dweik5, Hashim Syed6 and Omer Pamuk7, 1University Hospitals/ Case Western Reserve University, Cuyahoga Falls, OH, 2Case Western Reserve, University Hospitals, Cleveland Medical Center, Cleveland, OH, 3Case Western Reserve University, Westlake, OH, 4Case Western Reserve University/University Hospitals, Akron, OH, 5Cleveland Clinic Akron General Internal Medicine, Akron, OH, 6Case Western/ UH, Cleveland, 7University Hospitals Cleveland Medical Center/ Case Western Reserve University, Cleveland, OH

    Background/Purpose: Hydralazine-induced ANCA-associated vasculitis (HIAAV) is a rare clinical problem.  There is paucity of data regarding clinical features, treatment, and prognosis of hydralazine-induced AAV in…
  • Abstract Number: 0723 • ACR Convergence 2024

    Sociodemographic Factors Associated with Clinic Non-attendance and Unscheduled Emergency Care Episodes in ANCA-associated Vasculitis

    Warren RG James1, Corri Black1, Neil Basu2, Mark A Little3 and Rosemary Hollick1, 1University of Aberdeen, Aberdeen, United Kingdom, 2University of Glasgow, Glasgow, Scotland, United Kingdom, 3Trinity College Dublin, Dublin, Ireland

    Background/Purpose: Increasing evidence suggests that socioeconomic status drives differences in outcomes for people with rheumatic conditions. Individuals with complex, rare rheumatic conditions such as antineutrophil…
  • 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: 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: 2480 • ACR Convergence 2024

    Avacopan versus a Prednisone Taper in Patients with ANCA-Associated Vasculitis and Ear, Nose, or Throat Involvement in a Phase 3 Trial

    Robert Spiera1, Robert Lebovics2, Sarah Bray3, Rachel E. Gurlin4, David Jayne5 and Peter Merkel6, and ADVOCATE Study Group, 1Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 2Hackensack Meridian University Health System, Hackensack, NJ, 3Amgen Ltd, Cambridge, United Kingdom, 4Amgen Inc., Mountain View, CA, 5University of Cambridge, Cambridge, United Kingdom, 6University of Pennsylvania, Philadelphia, PA

    Background/Purpose: In the phase 3 ADVOCATE trial, 45.2% and 42.1% of patients in the avacopan and prednisone taper groups, respectively, had active ear, nose, or…
  • Abstract Number: 2526 • ACR Convergence 2024

    Development of the Hospital Emergency Advocacy & Treatment Kit (HEAT Kit) for Patients with Vasculitis

    Sarah Jones1, Pamela Squires2 and Donna McArthur3, 1Eosinophilic & Rare Disease Cooperative, Tucson, AZ, 2Eosinophilic & Rare Disease Cooperative, Tucson, 3Adjunct professor of nursing, Vanderbilt School of Nursing; Adjunct clinical professor, University of Arizona College of Nursing & Dept. of Neurology, Tucson, AZ

    Background/Purpose: All forms of vasculitis are rare with patients frequently experiencing poor or extended care in the hospital and ED settings. The ERDC founder experienced…
  • Abstract Number: 0724 • ACR Convergence 2024

    Incidence of ANCA-Associated Vasculitis in a Northern Spanish Health Region Between 2000-2023: A Populatio-based Study

    Fabricio Benavides1, Alba Herrero-Morant2, Salma Al Fazazi3, Vanesa Calvo-Rio4, Mónica Renuncio-García5, Adrián Martín-Gutiérrez6, Amparo Sánchez-López7, Claudia Poo-fernandez7, Clara Escagedo-Cagigas7, Maria Rodríguez-Vidriales1 and Ricardo Blanco-Alonso8, 1Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain, 2Hospital Universitario Marqués de Valdecilla, Ontinyent, Cantabria, Spain, 3Hospital Universitario Virgen de la Victoria, Málaga, Andalucia, Spain, 4Valdecilla Hospital, Santander, Cantabria, Spain, 5Division of Immunology, Hospital Universitario Marqués de Valdecilla, Immunopathology Group, IDIVAL, Santander, Spain, 6Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, 7Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, 8Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

    Background/Purpose: Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is a group of vasculitis that affect small vessels. Includes Granulomatosis with polyangiitis (GPA), Eosinophilic granulomatosis with…
  • Abstract Number: 1254 • ACR Convergence 2024

    The Impact of Eosinophilic Granulomatosis with Polyangiitis on the Health-Related Quality of Life of Patients and Their Ability to Work: Evidence from a Real-World Survey in Clinical Practice

    Robert Spiera1, Paul Dolin2, Anat Shavit2, Jennifer Rowell3, Chris Edmonds4, Josefine Persson5, Danuta Kielar6, Daniel Mascia7, James Siddall7, Tia Pennant7, Fritha Hennessy7 and Stephanie Y. Chen8, 1Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 2BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, 3Market Access and Pricing, AstraZeneca, Cambridge, England, United Kingdom, 4Market Access and Pricing, AstraZeneca, Gaithersburg, MD, 5Market Access and Pricing, AstraZeneca, Gothenburg, Sweden, 6Biopharmaceuticals Medicine, AstraZeneca, Cambridge, United Kingdom, 7Adelphi Real World, Bollington, United Kingdom, 8BioPharmaceuticals Medical, AstraZeneca, Gaithersburg, MD

    Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody-associated vasculitis that confers significant disease burden. This real-world study described the impact of…
  • 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: 2481 • ACR Convergence 2024

    General, Nervous System, Eye, and Skin Involvement in the Phase 3 Trial of Avacopan for the Treatment of ANCA-Associated Vasculitis

    Rula A. Hajj-Ali1, Duvuru Geetha2, Raashid Luqmani3, Christian Pagnoux4, Darcy Trimpe5, David Jayne6 and Peter Merkel7, and ADVOCATE Study Group, 1Cleveland Clinic, Cleveland, OH, 2Johns Hopkins University, Baltimore, MD, 3University of Oxford, Abingdon, United Kingdom, 4Mount Sinai Hospital, Toronto, ON, Canada, 5Amgen Inc., Thousand Oaks, CA, 6University of Cambridge, Cambridge, United Kingdom, 7University of Pennsylvania, Philadelphia, PA

    Background/Purpose: The most common types of ANCA-associated vasculitis, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), can affect a range of organs, including the kidneys,…
  • Abstract Number: 2651 • ACR Convergence 2024

    Vasculitis Associated with VEXAS Syndrome

    Megan Sullivan1, Kambiz Kalantari2, Carolyn Mead-Harvey3, Yael Kusne4, Mrinal Patnaik2, Abhishek Mangaonkar2, Ronald Go2, Daniel Montes2, Kaaren Reichard2, Horatiu Olteanu2, Melanie Bois2, Alexander Hines2, Julio Sartori-Valinotti5, Kenneth Warrington2 and Matthew Koster2, 1Mayo Clinic Arizona, Scottsdale, AZ, 2Mayo Clinic, Rochester, MN, 3Mayo Clinic, Scottsdale, AZ, 4Mayo Clinic, Phoenix, AZ, 5Mayo Clinic, Rohester, MN

    Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently defined clinical entity that causes hematologic and autoinflammatory symptoms. Since its initial description…
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