Abstracts tagged "ANCA associated vasculitis"

Abstract Number: 0533 • ACR Convergence 2021
Identification of a Novel Susceptibility Locus for Small Vessel Vasculitis with Autoantibodies Against Myeloperoxidase
Johanna Dahlqvist¹, Diana Ekman², Bengt Sennblad², Sergey Kozyrev³, Jessika Nordin², Åsa Karlsson³, Jennifer Meadows³, Erik Hellbacher¹, Solbritt Rantapaa-Dahlqvist⁴, Ewa Berglin⁵, Bernd Stegmayr⁵, Bo Baslund⁶, Øyvind Palm⁷, Hilde Haukeland⁷, Iva Gunnarsson⁸, Annette Bruchfeld⁸, Mårten Segelmark⁹, Sophie Ohlsson⁹, Aladdin Mohammad⁹, Anna Svärd¹⁰, Rille Pullerits¹¹, Hans Herlitz¹¹, Annika Söderbergh¹², Gerli Rosengren Pielberg³, Fabiana Farias³, Lina Hultin Rosenberg³, Matteo Bianchi³, Eva Muren³, Roald Omdal¹³, Roland Jonsson¹⁴, Maija-Leena Eloranta³, Lars Ronnblom³, Peter Söderkvist¹⁵, Ann Knight¹⁶, Per Eriksson¹⁵ and Kerstin Lindblad-Toh¹⁷, ¹Uppsala University and University Hospital, Uppsala, Sweden, ²Science for Life Laboratory, Uppsala University, Uppsala, Sweden, ³Uppsala University, Uppsala, Sweden, ⁴Ume University, Umea, Sweden, ⁵Umeå University and University Hospital, Umeå, Sweden, ⁶University of Copenhagen, Copenhagen, Denmark, ⁷Oslo University, Oslo, Norway, ⁸Karolinska Institute, Stockholm, Sweden, ⁹Lund University, Lund, Sweden, ¹⁰Uppsala University, Falun, Sweden, ¹¹Gothenburg University and Sahlgrenska Hospital, Gothenburg, Sweden, ¹²Dept. of Rheumatology, Örebro University Hospital, Örebro, Sweden, ¹³Stavanger University Hospital and University of Bergen, Stavanger, Norway, ¹⁴University of Bergen, Bergen, Norway, ¹⁵Linköping University, Linköping, Sweden, ¹⁶Rheumatology, Institute of Medical Sciences, Uppsala University, Uppsala, Sweden, ¹⁷Broad Institute of MIT and Harvard; and Uppsala University, Boston, MA
Background/Purpose: ANCA-associated vasculitides (AAV) are rare but aggressive autoimmune disorders. The pathogenesis of the disorders is complex and still poorly understood; only a few genetic…
Abstract Number: 0423 • ACR Convergence 2021
Urine and Plasma Complement Ba Levels During Flares of Nephritis in Patients with ANCA-Associated Vasculitis
Salem Almaani¹, Huijuan Song², Meshora Sthanithra², Christopher Toy², Anna Levesque², Lynn Fussner³, Alexa Meara³, Haikady Nagaraja², David Cuthbertson⁴, Nader Khalidi⁵, CURRY LEE MD KOENING⁶, Carol Langford⁷, Carol McAlear⁸, Larry Moreland⁹, Christian Pagnoux¹⁰, Philip Seo¹¹, Antoine Sreih¹², Kenneth Warrington¹³, Paul Monach¹⁴, Peter Merkel⁸, Brad Rovin³ and Dan Birmingham², ¹Ohio State University Medical Center, Columbus, OH, ²Ohio State university, Columbus, OH, ³Ohio State University Wexner Medical Center, Columbus, OH, ⁴University of South Florida, Tampa, FL, ⁵McMaster University, Hamilton, ON, Canada, ⁶LIMITED TO OFFICIAL UNIVERSITY DUTIES ONLY, Salt Lake City, UT, ⁷Cleveland Clinic, Moreland Hills, OH, ⁸University of Pennsylvania, Philadelphia, PA, ⁹University of Colorado, Denver, CO, ¹⁰Mount Sinai Hospital, Toronto, ON, Canada, ¹¹Johns Hopkins University, Baltimore, MD, ¹²Univeristy of Pennsylvania, Philadelphia, PA, ¹³Mayo Clinic, Rochester, Minnesota, ¹⁴Brigham and Women's Hospital, Boston, MA
Background/Purpose: The alternative complement pathway has been implicated in the pathogenesis of ANCA-associated vasculitis (AAV), however it is not clear whether activation of complement occurs…
Abstract Number: 0952 • ACR Convergence 2021
Outcomes of COVID-19 Infection in Patients with Primary Systemic Vasculitis and Polymyalgia Rheumatica: Results from the COVID-19 Global Rheumatology Alliance Physician Registry
Sebastian Sattui¹, Richard Conway², Michael Putman³, Andrea Seet⁴, Kaley Beins⁵, Catherine Hill⁶, David Liew⁷, Sarah Mackie⁸, Puja Mehta⁹, Lorna Neill¹⁰, Gimena Gomez¹¹, Maria Salinas¹², Federico Maldonado¹³, Henrique Moriz¹⁴, Samia Studart¹⁵, NAFICE ARAUJO¹⁶, Ann Knight¹⁷, Davide Rozza¹⁸, Luca Quartuccio¹⁹, Maxime Samson²⁰, Stephane Bally²¹, Alexandre Maria²², Pascal Chazerain²³, Rebecca Hasseli²⁴, Ulf Müller-Ladner²⁵, Bimba Franziska Hoyer²⁶, Reinhard Voll²⁷, Rita Pinheiro Torres²⁸, Mariana Luis²⁹, Sandra Lúcia Ribeiro³⁰, Samar Al Emadi³¹, Jeffrey Sparks³², Tiffany Hsu³³, Kristin D'Silva³⁴, Naomi Patel³⁴, Leanna Wise³⁵, Emily Gilbert³⁶, Maria Valenzuela-Almada³⁷, Ali Duarte-Garcia³⁸, Manuel Ugarte-Gil³⁹, Emily Sirotich⁴⁰, Jean Liew⁴¹, Jonathan Hausmann⁴², Paul Sufka⁴³, Suleman Bhana⁴⁴, Rebecca Grainger⁴⁵, Wendy Costello⁴⁶, Lindsay Jacobsohn⁴⁷, Zara Izadi⁴, Anja Strangfeld⁴⁸, Elsa Frazão Mateus⁴⁹, Kimme Hyrich⁵⁰, Laure Gossec⁵¹, Loreto Carmona⁵², Saskia Lawson-Tovey⁵⁰, Lianne Kearsley-Fleet⁵³, Martin Schaefer⁵⁴, Milena Gianfrancesco⁴, Pedro Machado⁵⁵, Zachary Wallace³⁴, Jinoos Yazdany⁴ and Philip Robinson⁵⁶, ¹Hospital for Special Surgery, New York, NY, ²St. James's University Hospital, Dublin, Ireland, ³Medical College of Wisconsin, Brookfield, WI, ⁴University of California San Francisco, San Francisco, CA, ⁵Vasculitis Foundation, Kansas City, MO, ⁶Queen Elizabeth Hospital, Woodville, Australia, ⁷Austin Health, Heidelberg, Australia, ⁸School of Medicine, University of Leeds, Leeds, United Kingdom, ⁹University College London, London, United Kingdom, ¹⁰PMR GCA Scotland, Perth, United Kingdom, ¹¹Research Unit Argentine Society of Rheumatology, Buenos Aires, Argentina, ¹²Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ¹³Sanatorio Güemes, Buenos Aires, Argentina, ¹⁴Hospital das Clinicas, Universidade Federal de Pernambuco, Pernambuco, Brazil, ¹⁵HOSPITAL GERAL DE FORTALEZA, Fortaleza, Brazil, ¹⁶Instituto de ASsistencia Medica ao Servidor Publico Estadual de São Paulo, São Paulo, Brazil, ¹⁷Rheumatology, Institute of Medical Sciences, Uppsala University, Uppsala, Sweden, ¹⁸Epidemiology Research Unit, Italian Society of Rheumatology, Rome, Italy, ¹⁹Clinical Rheumatology, Department of Medicine, University of Udine, Udine, Italy, ²⁰CHU Dijon Bourgogne, Hpital Franois Mitterrand, Dijon, Dijon, France, ²¹Nephrology and Dialysis Service, Metropole Savoie Hospital Center, Chambery, France, ²²Department of Internal Medicine and Multiorganic Diseases, Saint-Eloi University Hospital of Montpellier, Montpellier, France, ²³Department of Rheumatology and Internal Medicine, Diaconesses Croix Saint Simon Hospital, Paris, France, ²⁴Department of Rheumatology and Clinical Immunology, Campus Kerckhoff, Justus Liebig University Giessen, Bad Nauheim, Germany., Bad Nauheim, Germany, ²⁵JLU Giessen, Campus Kerckhoff, Dept. Rheum & Clin Immunol, Bad Nauheim, Germany, ²⁶Universittsklinikum Schleswig-Holstein, Kiel, Germany, ²⁷Department of Rheumatology and Clinical Immunology, University Medical Center, Faculty of Medicine, Albert-Ludwigs-University of Freiburg, Freiburg, Germany, ²⁸Rheumatology Service, Egas Moniz Hospital, Lisboa Occidental Hospital Centre, Lisbon, Portugal, ²⁹Department of Rheumatology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portuga, Coimbra, Portugal, ³⁰Universidade Federal do Amazonas, Amazonas, Brazil, ³¹Hamad medical corporation, Doha, Qatar, ³²Brigham and Women's Hospital, Boston, MA, ³³Brigham and Women's Hospital, Jamaica Plain, MA, ³⁴Massachusetts General Hospital, Boston, MA, ³⁵LAC+USC/Keck Medicine of USC, Pasadena, CA, ³⁶Mayo Clinic, Jacksonville, FL, ³⁷Division of Rheumatology, Mayo Clinic, Rochester, MN, ³⁸Mayo Clinic, Rochester, MN, ³⁹Hospital Guillermo Almenara Irigoyen, Essalud/Universidad Científica del Sur, Lima, Peru, ⁴⁰McMaster University, Hamilton, ON, Canada, ⁴¹Boston University, Boston, MA, ⁴²Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, ⁴³HealthPartners, Eagan, MN, ⁴⁴Crystal Run Health, Montvale, NJ, ⁴⁵University of Otago, Wellington, New Zealand, ⁴⁶Irish Children's Arthritis Network, Bansha, Ireland, ⁴⁷University of California San Francisco, Antioch, CA, ⁴⁸Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, ⁴⁹Liga Portuguesa Contra as Doenças Reumáticas (LPCDR), Lisbon, Portugal, ⁵⁰University of Manchester, Manchester, United Kingdom, ⁵¹Sorbonne Université and Hôpital Pitié-Salpêtrière, Paris, France, ⁵²Instituto de Salud Musculoesqueltica (InMusc), Madrid, Spain, ⁵³Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom, ⁵⁴German Rheumatism Research Center, Berlin, Germany, ⁵⁵Centre for Rheumatology & Department of Neuromuscular Diseases, University College London, London, United Kingdom, ⁵⁶University of Queensland School of Clinical Medicine, Brisbane, Queensland, Australia, Herston, Australia
Background/Purpose: Patients with primary systemic vasculitis (PSV) and polymyalgia rheumatica (PMR) may be at high risk for poor COVID-19 outcomes due to the treatments used,…
Abstract Number: 0425 • ACR Convergence 2021
Diagnostic Accuracy of Muscle MRI for Muscular Vasculitis in Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: A Pilot Study
Ryuta Inaba, Daisuke Waki, Keisuke Nishimura, Tomohiro Yoshida, Kaoru Mizukawa, Shintaro Yamamoto, Osamu Iri, Kohei Yo and Hiroyuki Murabe, Department of Endocrinology and Rheumatology, Kurashiki Central Hospital, Kurashiki, Japan
Background/Purpose: Histopathologic confirmation is the golden standard for diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), but sometimes can be difficult because of its invasion (e.g.…
Abstract Number: 0953 • ACR Convergence 2021
An International Delphi Exercise to Identify Items of Importance for Measuring Response to Treatment for ANCA-associated Vasculitis
Kaitlin Quinn¹, Sara Monti², Georgia Lanier³, Maria Bjork Viðarsdóttir⁴, Robin Christensen⁵, David R.W. Jayne⁶, Carol Langford⁷, Alfred Mahr⁸, Christian Pagnoux⁹, Gunnar Tomasson¹⁰ and Peter Merkel¹¹, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Washington, DC, ²University of Pavia, Pavia, Italy, ³n/a, Sherborn, MA, ⁴n/a, Reykjavik, Iceland, ⁵Section for Biostatistics and Evidence-Based Research, the Parker Institute, Bispebjerg and Frederiksberg Hospital, Copenhagen & Research Unit of Rheumatology, Department of Clinical Research, University of Southern Denmark, Copenhagen, Denmark, ⁶University of Cambridge, Cambridge, United Kingdom, ⁷Cleveland Clinic, Moreland Hills, OH, ⁸Kantonsspital St. Gallen, St.Gallen, Switzerland, ⁹Mount Sinai Hospital, Toronto, ON, Canada, ¹⁰University of Iceland, Reykjavik, Iceland, ¹¹University of Pennsylvania, Philadelphia, PA
Background/Purpose: ANCA-associated vasculitis (AAV) is characterized by fluctuating levels of disease activity. Randomized controlled trials (RCTs) in AAV have used multiple instruments to define active…
Abstract Number: 0426 • ACR Convergence 2021
Ocular Manifestations of ANCA-Associated Vasculitis
Mats Junek¹, Stephanie Garner¹, Lily Zhao¹, David Cuthbertson², Christian Pagnoux³, CURRY LEE MD KOENING⁴, Carol Langford⁵, Carol McAlear⁶, Paul Monach⁷, Larry Moreland⁸, Philip Seo⁹, Ulrich Specks¹⁰, Antoine Sreih¹¹, Kenneth Warrington¹⁰, Peter Merkel⁶ and Nader Khalidi¹, ¹McMaster University, Hamilton, ON, Canada, ²University of South Florida, Tampa, FL, ³Mount Sinai Hospital, Toronto, ON, Canada, ⁴LIMITED TO OFFICIAL UNIVERSITY DUTIES ONLY, Salt Lake City, UT, ⁵Cleveland Clinic, Moreland Hills, OH, ⁶University of Pennsylvania, Philadelphia, PA, ⁷Brigham and Women's Hospital, Boston, MA, ⁸University of Colorado, Denver, CO, ⁹Johns Hopkins University, Baltimore, MD, ¹⁰Mayo Clinic, Rochester, MN, ¹¹Univeristy of Pennsylvania, Philadelphia, PA
Background/Purpose: ANCA-associated vasculitides (AAV) are multisystem diseases that can have multiple ophthalmic manifestations. Although there are some data on ocular disease in granulomatosis with polyangiitis…
Abstract Number: 0954 • ACR Convergence 2021
The Association of Rituximab- vs. Cyclophosphamide-Based Remission Induction Strategies with Risk of End-Stage Renal Disease and Death in ANCA-Associated Vasculitis
Zachary Wallace¹, Xiaoqing Fu², Claire Cook³, Yuqing Zhang⁴, John Stone⁵ and Hyon K. Choi⁶, ¹Massachusetts General Hospital, Newton, MA, ²Massachusetts General Hospital, Boston, MA, ³Division of Rheumatology, Allergy, and Immunology, Mass General Hospital, Boston, MA, ⁴Division of Rheumatology, Allergy, and Immunology, Mass General Hospital, Harvard Medical School, Boston, MA, ⁵Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ⁶Massachusetts General Hospital, Lexington, MA
Background/Purpose: The RAVE trial established the non-inferiority of rituximab (RTX) vs cyclophosphamide (CYC) for remission induction of ANCA-Associated Vasculitis (AAV). Patients in RAVE were followed…
Abstract Number: 0427 • ACR Convergence 2021
Increased Risk of Severe Infections and Mortality in Patients with Newly Diagnosed Anti-neutrophil Cytoplasmic Antibody–associated Vasculitis: A Population-based Study
Kai Zhao¹, Hui Xie², John Esdaile³ and J. Antonio Avina-Zubieta², ¹Simon Fraser University, Coquitlam, BC, Canada, ²Arthritis Research Canada, Richmond, BC, Canada, ³Arthritis Research Canada, Vancouver, BC, Canada
Background/Purpose: Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are a group of multisystem inflammatory diseases of the small blood vessels. Infections are serious complications in AAV…
Abstract Number: 0955 • ACR Convergence 2021
The Effect of Treatment with the Complement C5a Receptor Inhibitor Avacopan on Health-Related Quality of Life in ANCA-Associated Vasculitis
Vibeke Strand¹, Pirow Bekker², Huibin Yue², David R.W. Jayne³ and Peter Merkel⁴, ¹Stanford University School of Medicine, Portola Valley, CA, ²ChemoCentryx, San Carlos, CA, ³University of Cambridge, Cambridge, United Kingdom, ⁴University of Pennsylvania, Philadelphia, PA
Background/Purpose: Avacopan, an oral C5a receptor inhibitor, was evaluated in ANCA-associated vasculitis (AAV). Efficacy and safety results were reported previously. Health-related quality of life (HRQoL)…
Abstract Number: 0428 • ACR Convergence 2021
ANCA Positivity and ANCA Associated Vasculitis in Patients with Rheumatoid Arthritis
Courtney O'Brien, Veena Ranganath and Tannaz Kermani, University of California Los Angeles, Los Angeles, CA
Background/Purpose: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) with rheumatoid arthritis (RA) has been reported rarely, and, studies have found ANCA positivity in RA. The…
Abstract Number: 1054 • ACR Convergence 2021
Metabolomics as an Innovative Tool for Cardiac Risk Stratification in Patients with Rheumatic Diseases: Results from a Pilot Study
Sorwe Mojtahed Poor¹, Lisa Hahnefeld², Frank Behrens³, Harald Burkhardt⁴, Michaela Köhm⁵, Geisslinger Gerd⁶ and Gurke Robert⁷, ¹University hospital Frankfurt Devision Rheumatology and Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Frankfurt am Main, Germany, ²pharmazentrum frankfurt/ZAFES, Institute of Clinical Pharmacology, Johann Wolfgang Goethe University, Frankfurt/Main, Germany, Frankfurt, Germany, ³Rheumatology & Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Fraunhofer Cluster Immune-Mediated Diseases (CIMD), Fraunhofer Cluster Immune-Mediated Diseases (CIMD), Frankfurt/Main, Germany, ⁴University hospital Frankfurt Devision Rheumatology and Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Frankfurt, Germany, ⁵University hospital Frankfurt Devision Rheumatology and Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Frankfurt, ⁶Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Frankfurt, Germany, ⁷pharmazentrum frankfurt/ZAFES, Institute of Clinical Pharmacology, Johann Wolfgang Goethe University, Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Frankfurt, Germany
Background/Purpose: Patients with rheumatic diseases (RMD) face an increased cardiovascular (CV) mortality compared to the general population. Still, risk stratification based on traditional cardiovascular risk…
Abstract Number: 0429 • ACR Convergence 2021
Causes and Characteristics of Death in ANCA-Associated Vasculitis – A Large, Real-Life, Contemporary Cohort Analysis
Guy Katz¹, Claire Cook², Xiaoqing Fu¹, Hyon K. Choi³ and Zachary S. Wallace⁴, ¹Massachusetts General Hospital, Boston, MA, ²Division of Rheumatology, Allergy, and Immunology, Mass General Hospital, Boston, MA, ³Massachusetts General Hospital, Lexington, MA, ⁴Harvard Medical School, Boston, MA
Background/Purpose: Despite improvements, patients with ANCA-associated vasculitis (AAV) have a higher risk of death than the general population. Contemporary treatment is associated with high remission…
Abstract Number: 1862 • ACR Convergence 2021
Clonal Hematopoiesis Across the Age Spectrum in Patients with Systemic Vasculitis
Kristina Wells¹, Fernanda Gutierrez-Rodrigues², Bhavisha Patel², Kaitlin Quinn³, Keith Sikora¹, Neal Young⁴ and Peter Grayson⁵, ¹National Institute of Arthritis, Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Washington, DC, ⁴National Heart, Lung, and Blood Institute (NHLBI), National Institues of Health, Bethesda, MD, ⁵National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: Clonal hematopoiesis (CH) is a pre-malignant state characterized by somatic mutations in hematologic precursor cells in genes associated with myeloid malignancies. Incidence of CH…
Abstract Number: 0430 • ACR Convergence 2021
Interstitial Lung Disease in ANCA-Associated Vasculitis: Associated Factors and Outcomes
Brett Doliner¹, Karen Rodriguez¹, Sydney Montesi¹, Xiaoqing Fu¹, Amita Sharma¹ and Zachary Wallace², ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital, Newton, MA
Background/Purpose: Despite treatment advances, ANCA-associated vasculitis (AAV) remains associated with excess morbidity and mortality. Interstitial lung disease (ILD) is an increasingly recognized manifestation of AAV…
Abstract Number: 1868 • ACR Convergence 2021
Neutrophil Extracellular Trap Formation in Patients with Vasculitis
Despina Michailidou¹, Runa Kuley¹, Tomas Mustelin², David Cuthbertson³, Peter Grayson⁴, Nader Khalidi⁵, CURRY LEE MD KOENING⁶, Carol Langford⁷, Carol McAlear⁸, Larry Moreland⁹, Christian Pagnoux¹⁰, Philip Seo¹¹, Ulrich Specks¹², Antoine Sreih⁸, Kenneth Warrington¹³, Paul Monach¹⁴, Peter Merkel¹⁵ and Christian Lood², ¹Division of Rheumatology, University of Washington, Seattle, WA, ²University of Washington, Seattle, WA, ³Health Informatics Institute, University of South Florida, South Florida, FL, ⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ⁵McMaster University, Hamilton, ON, Canada, ⁶LIMITED TO OFFICIAL UNIVERSITY DUTIES ONLY, Salt Lake City, UT, ⁷Cleveland Clinic, Moreland Hills, OH, ⁸Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ⁹Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹⁰Mount Sinai Hospital, Toronto, ON, Canada, ¹¹Johns Hopkins University, Baltimore, MD, ¹²Mayo Clinic, Rochester, MN, ¹³Mayo Clinic, Rochester, Minnesota, ¹⁴Brigham and Women's Hospital, Boston, MA, ¹⁵University of Pennsylvania, Philadelphia, PA
Background/Purpose: Neutrophil extracellular trap (NET) formation, the extrusion of chromatin and granular components from activated neutrophils, is essential in host defense. However, NET formation has…

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