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: 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: 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: 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…
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: 1609 • ACR Convergence 2024
Characteristics of a Cohort of Patients with Interstitial Lung Disease and ANCA Positivity in a University Hospital
Cristina Valero¹, Claudia Valenzuela², Elisa Martínez-Besteiro³, Patricia Quiroga Colina⁴, Arantzazu Alfranca⁵, Esther Vicente-rabaneda⁶, Santos Castañeda⁶ and Rosario García-Vicuña⁷, ¹Hospital de la Princesa, Madrid, Spain, ²ILD Unit, Pulmonology Department, Hospital Universitario de la Princesa, University Autonomade Madrid, Madrid, Spain, Madrid, Madrid, Spain, ³Pulmonology Service, Hospital Universitario de la Princesa, IIS-Princesa, Madrid, Spain, Madrid, Madrid, Spain, ⁴Division of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ⁵Immunology Service, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, Madrid, Madrid, Spain, ⁶Hospital Universitario de La Princesa, Madrid, Spain, ⁷Hospital Universitario de la Princesa, Madrid, Madrid, Spain
Background/Purpose: The prevalence of ANCA positivity at diagnosis of interstitial lung disease (ILD) ranges between 4-36% for anti-MPO and 2-4% for anti-PR3. ILD is more…
Abstract Number: 2484 • ACR Convergence 2024
Real-World Use of Avacopan for ANCA-Associated Vasculitis Beyond 52 Weeks
Julia Ford¹, Abdallah Geara², Duvuru Geetha³ and Ora Singer⁴, ¹University of Michigan, Ann Arbor, MI, ²University of Pennsylvania, Philadelphia, PA, ³Johns Hopkins University, Baltimore, MD, ⁴University of Michigan, Huntington Woods, MI
Background/Purpose: The landmark ADVOCATE trial, which led to the approval of avacopan (AVP) as adjunctive treatment of ANCA-associated vasculitis (AAV), treated patients with AVP for…
Abstract Number: 2690 • ACR Convergence 2024
ANCA IgG Promotion of Neutrophil Recruitment, Migration and Vascular Damage in ANCA Associated Vasculitis May Be Enhanced by Hypoxia
Noelle Pisacano, Amrita Dhutia, Stephen Rothery, Charles Pusey, Edwin Chilvers, Andrew Cowburn, Katharine Lodge and Maria Prendecki, Imperial College London, London, United Kingdom
Background/Purpose: Neutrophils are main mediators of disease in ANCA associated vasculitis (AAV). Pathogenic ANCA IgG on neutrophils promotes neutrophil adhesion and migration at the endothelium.…

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