Abstracts tagged "ANCA associated vasculitis"

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…
Abstract Number: 0725 • ACR Convergence 2024
Serum Glial Fibrillary Acidic Protein Could Assess Cross-sectional Vasculitis Activity by Reflecting Renal Involvement in Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitis
Lucy Eunju Lee¹, Taejun Yoon², Yong-Beom Park² and Sang-Won Lee², ¹Yonsei University, Seoul, South Korea, ²Yonsei University College of Medicine, Seoul, Republic of Korea
Background/Purpose: Glial fibrillary acidic protein (GFAP), an intermediate filament protein produced by astrocytes in the CNS, has been identified as a biomarker for disease activity…
Abstract Number: 1276 • ACR Convergence 2024
Therapeutic Drug Monitoring (TDM) of Rituximab to Predict Early B-Cell Repopulation in Children
Rana Alsulami¹, Bindiya Chugani², Joley Johnstone², Ann Yeh², Floris Loeff³, Linda Hiraki², Andrea Knight⁴, Deborah Levy² and Ruud Verstegen², ¹University of Toronto, Toronto, ON, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada, ³Sanquin Diagnostic Services, Amsterdam, Netherlands, ⁴Division of Rheumatology, The Hospital for Sick Children; Neurosciences and Mental Health, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada
Background/Purpose: Rituximab is increasingly used in pediatric inflammatory diseases, with dosing extrapolated from adult data due to a lack of pediatric-specific pharmacokinetic (PK) information. Children…
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: 2483 • ACR Convergence 2024
Efficacy and Safety of Avacopan over 12 Months in Japanese Patients with MPA/GPA: A Single-center Study
Yusuke Ushio¹, Hiromi Shimada², taichi miyagi³, Koichi Sugihara⁴, Mao Mizusaki⁵, Rina Mino⁵, Hayamasa yamaguchi⁵, Naoto Manabe⁵, Shusaku Nakashima² and Hiroaki Dobashi², ¹Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki, Kita District, Kagawa, Japan, ²Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan, ³Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, kidagun, Japan, ⁴Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Kagawa Prefecture, Japan, ⁵Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan
Background/Purpose: Avacopan, an oral C5a receptor inhibitor, was demonstrated to be an alternative to glucocorticoids (GC) in the treatment of microscopic polyangiitis (MPA)/granulomatosis with polyangiitis…
Abstract Number: 2689 • ACR Convergence 2024
Characterization of Alpha-1 Antitrypsin Function in ANCA-Associated Vasculitis
Lynn Fussner¹, Ivan Bilic², Carol McAlear³, David Cuthbertson⁴, Jie Cheng⁵, Elise Chen⁵, Markus Weiller², Ulrich Specks⁶ and Peter Merkel³, and for the Vasculitis Clinical Research Consortium, ¹The Ohio State University, Columbus, OH, ²Takeda Pharmaceuticals, Vienna, Austria, ³University of Pennsylvania, Philadelphia, PA, ⁴University of South Florida, Tampa, FL, ⁵Takeda Pharmaceuticals, Cambridge, MA, ⁶Mayo Clinic, Rochester, MN
Background/Purpose: Two separate genome-wide association studies demonstrated that polymorphisms in SERPINA1, encoding serine protease inhibitor alpha-1 antitrypsin (A1AT), are associated with increased risk of developing…

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