Abstracts tagged "ANCA associated vasculitis"

Abstract Number: 0715 • ACR Convergence 2024
Diffuse Alveolar Hemorrhage in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Revisited
Samuel Falde¹, Elif Ediboglu¹, Misbah Baqir¹, Rodrigo Cartin-Ceba², Fernando C. Fervenza¹, Ladan Zand¹, Matthew Koster¹, Kenneth Warrington¹ and Ulrich Specks¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Scottsdale, AZ
Background/Purpose: We sought to characterize the most recent cohort of patients presenting with DAH and AAV and to identify risk factors for respiratory failure and…
Abstract Number: 0735 • ACR Convergence 2024
ANCA-associated Vasculitis (AAV) and Long-term Risk of Cardiovascular Disease: Large-scale Propensity-matched Global Retrospective Cohort Study
Sebastian Klapa¹, Henry Nording², Marlene Ludwig³, Sabrina Arnold⁴, Khalaf Kridin⁵, Anja Staehle⁶, Ralf Ludwig⁷ and Peter Lamprecht⁴, ¹University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, ²University of Lübeck, Cardioimmunology Research Group, Lübeck, Germany, ³Independent Researcher, Groß Grönau, Germany, ⁴University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, ⁵5. Bar-Ilan University, Azrieli Faculty of Medicine, Safed, Israel, ⁶University of Lübeck, Department of Rheumatology and Immunology, Lübeck, Germany, ⁷University of Lübeck, Lübeck Institute of Experimental Dermatology, Lübeck, Germany
Background/Purpose: In spite of significantly improved therapy during the last years, long-term morbidity and mortality has remained high in ANCA-associated vasculitis (AAV). The main causes…
Abstract Number: 1600 • ACR Convergence 2024
Glucocorticoids versus Glucocorticoids Plus Cyclophosphamide in Eosinophilic Granulomatosis with Polyangiitis Without Poor-Prognosis Factors: A Target Trial Emulation Study
Boris Sorin¹, Matthias Papo¹, Renato Alberto Sinico², Vítor Silvestre Teixeira³, Nils Venhoff⁴, Maria-Letizia Urban⁵, Michele Iudici⁶, Juliane Mahrhold⁷, Francesco Locatelli⁸, Giulia Cassone⁹, Franco Schiavon¹⁰, Benjamin Seeliger¹¹, Thomas Neumann¹², Claudia Feder¹³, Claus Kroegel¹⁴, Matthieu Groh¹⁵, Chiara Marvisi¹⁶, Maxime Samson¹⁷, Thomas Barba¹⁸, David Jayne¹⁹, Arianna Troilo²⁰, Jens Thiel²⁰, Bernhard Hellmich²¹, Sara Monti²², Carlomaurizio Montecucco²³, Carlo Salvarani²⁴, Jean-Emmanuel Kahn²⁵, Bernard Bonnotte²⁶, Cécile-Audrey Durel²⁷, Xavier Puéchal²⁸, Luc Mouthon²⁹, Loïc Guillevin³⁰, Giacomo Emmi³¹, Augusto Vaglio³², Raphael Porcher³³ and Benjamin Terrier³⁴, and the French Vasculitis Study Group (FVSG) and the European EGPA Study Group (EESG), ¹Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris, Paris, France, ²Nephrology and Dialysis Unit, IRCCS Humanitas Research Hospital, Milan, Italy, ³Department of Rheumatology, Faro Hospital, Algarve, Portugal, ⁴Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Paris Cité University, Assistance Publique – Hôpitaux de Paris and Division of rheumatology, Geneva University Hospitals and University of Geneva, Geneva, Switzerland, Paris, France, ⁷Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabtologyy and Immunology, ERN-RITA Reference Center, Vasculitis-Center Tübingen-Kirchheim, Medius Kliniken Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, ⁸Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy, ⁹Clinical and Experimental Medicine PhD Program, Azienda USL-IRCCS di Reggio Emilia and Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁰Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ¹¹Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany, ¹²Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany and Division of Rheumatology, Immunology and Rehabilitation, Kantonsspital St Gallen, St Gallen, Switzerland, Jena, Germany, ¹³Department of Rheumatology, Clinic of Internal Medicine III, Jena University Hospital, Jena, Germany, ¹⁴Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany, ¹⁵Foch, Suresnes, France, ¹⁶Rheumatology Unit Universita di Modena and Reggio Emilia, Modena, Italy, ¹⁷Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France, ¹⁸Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, ¹⁹University of Cambridge, Cambridge, United Kingdom, ²⁰Department of Rheumatology and Clinical Immunology, Medical Center – University of Freiburg, Faculty of Medicine, Freiburg, Germany, ²¹Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany, ²²IRCCS Istituto Auxologico Italiano, Milan, Italy, ²³IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ²⁴Azienda USL-IRCCS di Reggio Emilia and University of Modena and Reggio Emilia, Reggio Emilia, Italy, Reggio Emilia, Italy, ²⁵Department of Internal Medicine, Ambroise Paré Hospital, Assistance Publique - Hôpitaux de Paris, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France, Boulogne Billancourt, France, ²⁶Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France., Dijon, France, ²⁷Edouard Herriot University Hospital, Lyon, France, ²⁸National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ²⁹Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l'Est et de l'Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ³⁰National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ³¹Department of Medical, Surgery and Health Sciences, University of Trieste, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy, ³²Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Florence, Italy, ³³Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, Ile-de-France, France, ³⁴Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel vasculitis characterized by asthma, blood and tissue eosinophilia and vasculitis affecting multiple organs. According to the…
Abstract Number: 2180 • ACR Convergence 2024
The Association Between Age at Diagnosis and Patient-Reported Outcomes in Patients with Vasculitis
Jessica Bloom¹, Kaci Pickett-Nairne², Lori Silveira², David Cuthbertson³, Carol McAlear⁴, Peter Merkel⁴ and Karen James⁵, and the Vasculitis Clinical Research Consortium, ¹University of Colorado, Denver, CO, ²University of Colorado, Aurora, CO, ³University of South Florida, Tampa, FL, ⁴University of Pennsylvania, Philadelphia, PA, ⁵University of Utah, Salt Lake City, UT
Background/Purpose: Vasculitis is a chronic autoimmune condition that significantly impacts patients’ quality of life. It is unknown whether the degree of this impact varies based…
Abstract Number: 2493 • ACR Convergence 2024
Mortality in Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: Study from a Single University Hospital
Fabricio Benavides¹, Alba Herrero-Morant², Diana Prieto-Peña³, Vanesa Calvo-Rio⁴, Salma Al Fazazi⁵, Adrián Martín-Gutiérrez⁶, Mónica Renuncio-García⁷, Amparo Sánchez-López⁸, Claudia Poo-fernandez⁸, Clara Escagedo-Cagigas⁸, Maria Rodríguez-Vidriales¹ and Ricardo Blanco-Alonso⁹, ¹Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain, ²Hospital Universitario Marqués de Valdecilla, Ontinyent, Cantabria, Spain, ³Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Cantabria, Spain, ⁴Valdecilla Hospital, Santander, Cantabria, Spain, ⁵Hospital Universitario Virgen de la Victoria, Málaga, Andalucia, Spain, ⁶Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, ⁷Division of Immunology, Hospital Universitario Marqués de Valdecilla, Immunopathology Group, IDIVAL, Santander, Spain, ⁸Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, ⁹Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) includes Granulomatosis with polyangiitis (GPA), Microscopic polyarteritis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA). Due to clinical,…
Abstract Number: 0716 • ACR Convergence 2024
Outcomes of COVID-19 in Patients with ANCA-Associated Vasculitis (AAV)
Husam El Sharu¹, Teeba Mubaydeen² and Maria Romero Noboa³, ¹East Carolina University, Greenville, NC, ²King Hussein Cancer Center, Amman, Jordan, ³University of Alabama at Birmingham, Chicago, IL
Background/Purpose: Patients with AAVs are typically on immunosuppression medications that might increase their risk of severe infections. COVID-19 has been associated with severe pulmonary manifestations…
Abstract Number: 0823 • ACR Convergence 2024
A Randomized, Double-Blind, Placebo-Controlled Trial of Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis with Polyangiitis
Carol Langford¹, Nader Khalidi², Jason Springer³, Marcia Friedman⁴, Bernhard Hellmich⁵, Christian Pagnoux⁶, Natasha Dehghan⁷, Ora Singer⁸, Curry Koening⁹, Yih Chang Lin¹⁰, Paul Monach¹¹, Larry Moreland¹², Aurore Fifi-Mah¹³, Oliver Flossmann¹⁴, Lindsy Forbess¹⁵, Peter Lanyon¹⁶, Eamonn Molloy¹⁷, Ulrich Specks¹⁸, Robert Spiera¹⁹, Elaine Yacyshyn²⁰, Carol McAlear²¹, Cristina Burroughs¹⁰, Rachel Jones²², Rennie Rhee²¹, Rula A. Hajj-Ali²³, Kenneth Warrington¹⁸, David Cuthbertson¹⁰, Jeffrey Krischer¹⁰, David Jayne²² and Peter Merkel²¹, and the Vasculitis Clinical Research Consortium and the European Vasculitis Society, ¹Cleveland Clinic, Moreland Hills, OH, ²McMaster University, Hamilton, ON, Canada, ³Vanderbilt University Medical Center, Franklin, TN, ⁴Oregon Health and Science University, Portland, OR, ⁵Medius Kliniken, Kirchheim unter Teck, Germany, ⁶Mount Sinai Hospital, Toronto, ON, Canada, ⁷University of British Columbia - Vancouver, Vancouver, BC, Canada, ⁸University of Michigan, Huntington Woods, MI, ⁹University of Texas Dell Medical School, Austin, TX, ¹⁰University of South Florida, Tampa, FL, ¹¹VA Boston Healthcare System, Boston, MA, ¹²University of Colorado, Denver, CO, ¹³University of Calgary, Calgary, AB, Canada, ¹⁴Royal Berkshire Hospital, Reading, United Kingdom, ¹⁵Cedars-Sinai Medical Center, Los Angeles, CA, ¹⁶University of Nottingham, Nottingham, United Kingdom, ¹⁷St Vincent's University Hospital, Dublin, Ireland, ¹⁸Mayo Clinic, Rochester, MN, ¹⁹Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, ²⁰University of Alberta, Edmonton, AB, Canada, ²¹University of Pennsylvania, Philadelphia, PA, ²²University of Cambridge, Cambridge, United Kingdom, ²³Cleveland Clinic, Cleveland, OH
Background/Purpose: Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis associated with frequent relapses. Following encouraging results from an open-label study, a randomized, double-blind, placebo-controlled trial…
Abstract Number: 1601 • ACR Convergence 2024
Changing Spectrum of Systemic Therapies for Eosinophilic Granulomatosis with Polyangiitis from 2006-2023
Lynn Fussner¹, Christian Pagnoux², David Cuthbertson³, Thomas Corbridge⁴, Nader Khalidi⁵, Curry Koening⁶, Carol Langford⁷, Carol McAlear⁸, Paul Monach⁹, Larry Moreland¹⁰, Rennie Rhee⁸, Philip Seo¹¹, Anat Shavit¹², Jared Silver¹³, Ulrich Specks¹⁴, Kenneth Warrington¹⁴, Michael Wechsler¹⁵ and Peter Merkel⁸, and for the Vasculitis Clinical Research Consortium, ¹The Ohio State University, Columbus, OH, ²Mount Sinai Hospital, Toronto, ON, Canada, ³University of South Florida, Tampa, FL, ⁴US Value Evidence and Outcomes, GSK, Durham, NC, ⁵McMaster University, Hamilton, ON, Canada, ⁶University of Texas Dell Medical School, Austin, TX, ⁷Cleveland Clinic, Moreland Hills, OH, ⁸University of Pennsylvania, Philadelphia, PA, ⁹VA Boston Healthcare System, Boston, MA, ¹⁰University of Colorado, Denver, CO, ¹¹Johns Hopkins University, Baltimore, MD, ¹²BioPharmaceuticals Medical, AstraZeneca, Cambridge, United Kingdom, ¹³US Medical Affairs - Respiratory, GSK, Durham, ¹⁴Mayo Clinic, Rochester, MN, ¹⁵National Jewish Health, Denver, CO
Background/Purpose: The emergence of biologic therapies targeting eosinophils via the IL-5 pathway provided new options for treatment in eosinophilic granulomatosis with polyangiitis (EGPA). There has…
Abstract Number: 2181 • ACR Convergence 2024
Large Pediatric Cohort with ANCA-associated Vasculitis at a Single Institution: Patient Characteristics, Clinical Course, and Outcomes
Jessica Bloom¹, Anna Monley², Sarah Reingold³, Robert Fuhlbrigge¹ and Peter Merkel⁴, ¹University of Colorado, Denver, CO, ²University of Colorado, Aurora, CO, ³Denver Health, Denver, CO, ⁴University of Pennsylvania, Philadelphia, PA
Background/Purpose: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is an understudied, chronic inflammatory disease in children with significant morbidity and mortality. There are few large pediatric cohorts…
Abstract Number: 2494 • ACR Convergence 2024
Clinical Characteristics, Predictors for Mortality and Comparison of the Birmingham Vasculitis Activity Score and the Five-Factor Score on Survival in ANCA-associated Vasculitis in Hong Kong : A Multi-centre Retrospective Cohort Study
Ka Ho Yeung and Kit Yu Young, Caritas Medical Centre, Hong Kong, Hong Kong
Background/Purpose: To describe the clinical profile and predictors of mortality of AAV patients in Hong Kong. To compare the accuracy of the latest Five-factor Score…
Abstract Number: 0717 • ACR Convergence 2024
Associations Between Calendar Time and Age at Diagnosis with Mortality in a National Cohort of Veterans with ANCA-Associated Vasculitis from 2001-2020
Jennifer Hanberg¹, Svetlana Eden², Amy Anderson-Mellies², Suman Kundu², Matthew Freiberg², Zachary Wallace³ and Evelyn Hsieh⁴, and the Veterans Aging Cohort Study Project Team, ¹Brigham and Women's Hospital, Boston, MA, ²Vanderbilt University Medical Center and Tennessee Valley VA, Nashville, TN, ³Massachusetts General Hospital, Newton, MA, ⁴Yale School of Medicine / VA Connecticut Healthcare System, West Haven, Connecticut, New Haven, CT
Background/Purpose: It is not clear whether advances in ANCA-associated vasculitis (AAV) management between 2001-2020 have translated to improvement in population-level outcomes, including among older adults.…
Abstract Number: 0824 • ACR Convergence 2024
The Effect of Intranasal Niclosamide on Nasal Symptoms in Patients with ENT Manifestations of ANCA-Associated Vasculitis (AAV): Post Hoc Analysis of Subset of Patients Recruited to the PROTECT-V Trial
Benjamin Lim¹, Wendi Qian¹, Francis Dowling², Michael Chen-Xu¹, Alan Salama³ and Rona Smith⁴, ¹University of Cambridge, Cambridge, ²Cambridge University Hospitals NHS Foundation Trust, Cambridge, ³University College London, London, ⁴University of Cambridge, Cambridge, United Kingdom
Background/Purpose: Ear, nose and throat (ENT) manifestations are common in ANCA associated vasculitis (AAV), particularly the PR3 (proteinase 3) sub type. There is unmet need…
Abstract Number: 1603 • ACR Convergence 2024
Antineutrophil Cytoplasmic Autoantibody Levels in Patients in the Avacopan Phase 3 Trial
Frank B. Cortazar¹, Duvuru Geetha², Salem Almaani³, Christina Song⁴, Tomasz M. Wilmanski⁴, Alana M. Bozeman⁴, Peter Merkel⁵ and David Jayne⁶, and ADVOCATE Study Group, ¹New York Nephrology Vasculitis and Glomerular Center, Saint Peter’s Hospital-Albany, Albany, NY, ²Johns Hopkins University, Baltimore, MD, ³The Ohio State University Medical Center, Columbus, OH, ⁴Amgen Inc., Thousand Oaks, CA, ⁵University of Pennsylvania, Philadelphia, PA, ⁶University of Cambridge, Cambridge, United Kingdom
Background/Purpose: The utility of measuring serial antineutrophil cytoplasmic autoantibody (ANCA) levels to guide treatment in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) is controversial.…
Abstract Number: 2188 • ACR Convergence 2024
Parent Verses Patient Perspectives of Health-Related Quality of Life Among Children with Vasculitis
Clare Peckenpaugh¹, Aimee Hersh², Sara Stern², Erin Treemarcki², Peter Merkel³ and Karen James², ¹University of Utah Department of Pediatrics, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT, ³University of Pennsylvania, Philadelphia, PA
Background/Purpose: Vasculitis is a group of chronic, relapsing diseases that can lead to significant morbidity due to both disease and treatment, impacting patients’ health related…
Abstract Number: 2495 • ACR Convergence 2024
Diffuse Alveolar Hemorrhage in Patient with ANCA-associated Vasculitis AndInterstitial Lung Disease
Elif Ediboglu¹, Samuel Falde¹, Misbah Baqir¹, Rodrigo Cartin-Ceba² and Ulrich Specks¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Scottsdale, AZ
Background/Purpose: The association of interstitial lung disease (ILD) and microscopic polyangiitis (MPA) is increasingly recognized. The implications of ILD as it relates to clinical outcomes…

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