Abstracts tagged "ANCA associated vasculitis"

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…
Abstract Number: 0726 • ACR Convergence 2024
Management of ANCA Vasculitis-Associated Orbital Inflammatory Disease: A Systematic Literature Review
Emma Neary¹, Katie Healey², Marie Clements-Baker³, Jean-Paul Makhzoum⁴ and Arielle Mendel⁵, ¹McGill University, Montreal, QC, Canada, ²Memorial University of Newfoundland, St. John's, NL, Canada, ³Queen's University, Kingston, ON, Canada, ⁴Vasculitis Clinic, Canadian Network for Research on Vasculitides, Hopital du Sacre-Coeur de Montreal, Montreal, QC, Canada, ⁵McGill University Health Centre, Montreal, QC, Canada
Background/Purpose: Ocular manifestations are common in ANCA-associated vasculitides (AAV), with orbital inflammatory disease (OID) representing a significant subset. Despite being a vision-threatening complication, OID remains…
Abstract Number: 1294 • ACR Convergence 2024
Using Case-Based Continuing Education to Identify and Address Knowledge and Behavior Gaps in ANCA-associated Vasculitis
Bharati Hegde, Jillian Scavone, Katie Robinson and Robert Esgro, Vindico Medical Education, West Deptford, NJ
Background/Purpose: Given the rarity and non-specific symptoms associated with ANCA-associated vasculitis (AAV), rheumatology clinicians are challenged to use best practices for patient recognition and management,…
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…

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