Abstracts tagged "Vasculitis"

Abstract Number: 2404 • ACR Convergence 2023
Subclinical Giant Cell Arteritis in Polimialgia Rheumatica: A Biomarker of High Relapse Risk
Eugenio De Miguel¹, Rositsa Karalilova², PIERLUIGI MACCHIONI³, Cristina Ponte⁴, Edoardo Conticini⁵, Alessandro Tomelleri⁶, Sara Monti⁷, Irene Monjo¹, Zguro Batalov², Giulia Klinowski⁸, Paolo Falsetti⁹, David Kane¹⁰, Corrado Campochiaro¹¹ and Alojzija Hocevar¹², ¹Hospital Universitario La Paz, Madrid, Spain, ²Medical University of Plovdiv, University Hospital Kaspela, Plovdiv, Bulgaria, ³Azienda USL -IRCCS di Reggio Emilia, Reggio Emilia, Italy, ⁴Department of Rheumatology, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon, Portugal; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal, ⁵Tallaght University Hospital, Dublin, Ireland, ⁶Unit of Immunology, Rheumatology, Allergy and Rare Diseases, San Raffaele Scientific Institute, Milano, Italy, ⁷Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy, ⁸IRCCS-S.Maria Nuova, Reggio Emilia, Italy, ⁹San Raffaele Scientific Institute, Siena, Italy, ¹⁰Tallaght University Hospital & Trinity College Dublin, Dublin, Ireland, ¹¹IRCCS San Raffaele Hospital, Unit of Immunology, Rheumatology, Allergy and Rare Disease. Vita-Salute San Raffaele University, Milan, Italy, ¹²Department of Rheumatology, Universitiy Medical Centre Ljubljana, Ljubljana, Slovenia
Background/Purpose: Around 20% of polymyalgia rheumatica (PMR) patients without clinical symptoms of giant cell arteritis (GCA) have subclinical GCA by image or byopsy1. However, there…
Abstract Number: 2491 • ACR Convergence 2023
Alignment Between the Novel 2022 ACR/EULAR Classification Criteria for ANCA-associated Vasculitis (AAV), Clinical Diagnosis and Organ Manifestations in a European AAV Cohort
Stefan Krämer¹, Thomas Rauen¹, Kristian Vogt¹, Teresa Anslinger¹, Martin Busch², Tobias Schmitt³, Raoul Bergner⁴, Sebastian Mosberger⁴ and Thomas Neumann⁵, ¹RWTH, University Hospital Aachen, Department of Nephrology and Rheumatology, Aachen, Germany, ²University Hospital Jena, Friedrich-Schiller University, Department of Internal Mediciine III, Jena, Germany, ³University Hospital Jena, Friedrich Schiller University, Department of Nephrology, Jena, Germany, ⁴Municipal Hospital Ludwigshafen, Department of Internal Medicine A, Nephrology and Rheumatology, Ludwigshafen, Germany, ⁵Cantonal Hospital St. Gallen, Department of Rheumatology, St. Gallen, Switzerland
Background/Purpose: In 2022, ACR and EULAR proposed new classification criteria for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) based on a numerical item scoring…
Abstract Number: 0688 • ACR Convergence 2023
Efficacy and Safety Experience with Avacopan Beyond 52 Weeks in the Early Access Program (EAP)
Federico Alberici¹, Carlo Salvarani², Christine Chan³, Achim Obergfell⁴ and Tamara Popov⁴, ¹Universita degli Studi di Brescia, Brescia, Italy, ²Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ³CSL Vifor, Redwood City, CA, ⁴CSL Vifor, Glattbrugg, Switzerland
Background/Purpose: Avacopan, a selective C5aR1 inhibitor, has demonstrated efficacy and safety over 52 weeks in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, efficacy and…
Abstract Number: 0709 • ACR Convergence 2023
Survival of Adults with IgA Vasculitis
Alojzija Hocevar¹, JAKA OSTROVRŠNIK², vESNA JURČIĆ³, Matija Tomšič⁴ and Ziga Rotar⁵, ¹Department of Rheumatology, Universitiy Medical Centre Ljubljana, Ljubljana, Slovenia, ²UKC Ljubljana, Ljubljana, Slovenia, ³University of Ljubljana, Medical Faculty, Institute of Pathology, Ljubljana, Slovenia, ⁴Department of Rheumatology, University Medical Centre LJubljana, Ljubljana, Slovenia, ⁵University Medical Centre Ljubljana, Ljubljana, Slovenia
Background/Purpose: Data on survival of adults with IgA vasculitis (IgAV) are scarce. The aim of our study was to estimate for the first time the…
Abstract Number: 1248 • ACR Convergence 2023
Thrombotic Manifestations in Pediatric Behçet Disease Patients: A Multicentre Comparative Study from EUROFEVER Registry
Maria Vincenza Mastrolia¹, Caterina Matucci-Cerinic², Seza Ozen³, Ozgur Kasapcopur⁴, Carla Gaggiano⁵, Isabelle Kone-Paut⁶, Luca Cantarini⁵, Perrine Dusser⁷, Ümmüşen Kaya-Akça⁸, Mehmet Yildiz⁹, Juergen Brunner¹⁰, Giovanni Filocamo¹¹, Romina Gallizzi¹², Antonella Insalaco¹³, Serena Pastore¹⁴, Donato Rigante¹⁵, Judith Sanchez-Manubens¹⁶, Elena Tsitsami¹⁷, Marco Gattorno¹⁸ and Gabriele Simonini¹⁹, ¹NEUROFARBA Department, University of Florence, Florence, Italy, ²DINOGMI, University of Genoa; IRCCS Istituto Giannina Gaslini, UOC Rheumatology and autoinflammatory diseases, Genova, Italy, ³Hacettepe University Medical Faculty, Ankara, Turkey, ⁴Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, ⁵Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy, ⁶Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAIA, Bicêtre Hospital, APHP, University of Paris Saclay, Paris, France, ⁷Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, ⁸Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁹Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Instanbul, Turkey, ¹⁰Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ¹¹Pediatric Immunorheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, MIlan, Italy, ¹²Department of Medical of Health Sciences, Magna Graecia University, Catanzaro, Italy, ¹³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ¹⁴Institute for Maternal and Child Health, RCCS “Burlo Garofolo", Trieste, Italy, ¹⁵Department of Life Sciences and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy, ¹⁶Pediatric Rheumatology, Section Pediatric Service, Parc Taulí Sabadell University Hospital, Institute for Research and Innovation I3PT, Sant Joan de Deu Hospital, Autonomous University of Barcelona, Barcelona, Spain, ¹⁷Pediatric Rheumatology Unit, 1st Department of Pediatrics, Children's Hospital "Aghia Sophia", University of Athens, Athens, Greece, ¹⁸UOC Reumatologia e Malattie Autoinfiammatorie, Genoa, Italy, ¹⁹Rheumatology Unit, ERN ReConnet Center, Meyer Children's Hospital IRCCS, Florence Italy. NeuroFARBA Department, University of Florence, Florence, Italy
Background/Purpose: Arterial and venous thrombosis occurs in 6.6 to 38.4% of pediatric Behçet disease (BD) and cerebral sinus is one of the most involved districts.…
Abstract Number: 1565 • ACR Convergence 2023
Baseline Vascular Ultrasound of Polymyalgia Rheumatica Patients at Time of Diagnosis Predicts Clinical Outcomes at 3 Months
Sharon Cowley¹, Colm Kirby², Patricia Harkins³, Richard Conway⁴ and David Kane⁵, ¹Tallaght University Hospital, Dublin, Dublin, Ireland, ²Tallaght University Hospital, Dublin, Ireland, ³St James's Hospital, Dublin, Ireland, ⁴Trinity College Dublin, Dublin, Ireland, ⁵Tallaght University Hospital & Trinity College Dublin, Dublin, Ireland
Background/Purpose: It has been reported that up to a quarter of patients with polymyalgia rheumatica (PMR) have subclinical giant cell arteritis (GCA). It is currently…
Abstract Number: 1941 • ACR Convergence 2023
Identification of VEXAS Syndrome in Mexican Patients with Inflammatory and Hematologic Manifestations
Eduardo Martin-Nares¹, Beatriz E. Sánchez-Hernández², Peter Grayson³, Jose Crispin¹, Daniel Montante-Montes de Oca², Andrea Hinojosa-Azaola¹, Gabriela Hernandez-Molina¹, Elia Apodaca⁴, Emma M. Groarke⁵, Jesús Delgado-de la Mora², Silvia Méndez-Flores⁶, Armando Gamboa-Domínguez², Bhavisha Patel⁷ and Marcela Ferrada⁸, ¹Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ²Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ³National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD, ⁴Department of Oncology and Hematology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ⁵Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health (NIH), Bethesda, MD, ⁶Department of Dermatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ⁷National Institutes of Health, Beltsville, MD, ⁸National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: There is limited information on VEXAS syndrome in the Latin-American population. We aimed to identify UBA1 mutations causing VEXAS syndrome in Mexican patients presenting…
Abstract Number: 2406 • ACR Convergence 2023
Risk of Large Vessel Complications in Patients with Giant Cell Arteritis, a Population-based Study
Mohanad Elfishawi¹, Mahmut Kaymakci¹, Sara Achenbach², Cynthia Crowson¹, Tanaz Kermani³, Cornelia M. Weyand⁴, Matthew Koster¹ and Kenneth Warrington¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Rochester, MN, Rochester, MN, ³University of California Los Angeles, Los Angeles, CA, ⁴Mayo Clinic School of Medicine and Stanford University, Rochester, MN
Background/Purpose: Large vessel (LV) complications are known to occur in patients with giant cell arteritis (GCA). The magnitude of risk compared to the general population…
Abstract Number: 2492 • ACR Convergence 2023
The Impact of Neurologic Involvement in ANCA-Associated Vasculitis on Self-Reported Health-Related Quality of Life
Rula Hajj-Ali¹, Chao Zhang², Renee Borchin³, Jenn Gordon⁴, David Cuthbertson³, Carol McAlear⁵, Christine Yeung⁵, David Badenoch⁶, Cristina Burroughs³ and Peter Merkel⁵, ¹Cleveland Clinic, Cleveland, OH, ²Cleveland Clinic Foundation, Cleveland, OH, ³University of South Florida, Tampa, FL, ⁴Vasculitis Patient Powered Research Network (VPPRN), Philadelphia, PA, ⁵University of Pennsylvania, Philadelphia, PA, ⁶Vasculitis Patient Powered Research Network (VPPRN), Los Angeles, CA
Background/Purpose: Neurologic involvement (NI) is a common manifestation in patients with ANCA-associated vasculitis (AAV) and can lead to chronic pain and disability. This project assessed…
Abstract Number: 060 • 2023 Pediatric Rheumatology Symposium
Are the Levels of Cytokines Good Biomarkers for Smoldering Disease Activity in Childhood-Takayasu Arteritis?
Gleice Clemente¹, Maria Teresa Terreri², Bruno Gualano³, Clovis Silva⁴ and Alexandre Wagner De Souza¹, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²UNIFESP, São Paulo, Brazil, ³Universidade de São Paulo, São Paulo, ⁴Universidade de São Paulo, São Paulo, Brazil
Background/Purpose: Biomarkers for disease activity in adult Takayasu arteritis (TA) have been studied exhaustively, but there are inconsistencies among the studies (1). Childhood-TA (c-TA) differs…
Abstract Number: 061 • 2023 Pediatric Rheumatology Symposium
Variation in Treatment Approaches to IVIG- Refractory Kawasaki Disease (KD) Among Pediatric Rheumatologists: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) Treatment of Refractory KD Survey
Daniel Ibanez¹, Bianca Lang², Ali Yalcindag³, Linda Wagner-Weiner⁴, Julia Shalen⁵, Kenneth Schikler⁶, Shoghik Akoghlanian⁷, Hulya Bukulmez⁸, Kristen Hayward⁹, Sivia Lapidus¹⁰, Andrea Ramirez¹¹, Robert Sundel¹, Cagri Yildirim-Toruner¹² and CARRA Registry Investigators¹³, ¹Boston Children's Hospital, Boston, MA, ²Dalhousie University - Halifax, Halifax, NS, Canada, ³Hasbro Children's Hospital, Milton, MA, ⁴The University of Chicago, Chicago, IL, ⁵Johns Hopkins University, Baltimore, MD, ⁶University of Louisville School of Medicine Norton Children's Hospital, Louisville, KY, ⁷Nationwide Children's Hospital, Columbus, OH, ⁸MetroHealth Medical Center, Case Western Reserve, Cleveland, OH, ⁹Seattle Children's Hospital/University of Washington School of Medicine, Seattle, WA, ¹⁰Department of Pediatrics, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ, ¹¹Baylor College of Medicine, Houston, TX, ¹²Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ¹³CARRA, Washington, DC
Background/Purpose: Kawasaki disease (KD) is the leading cause of acquired heart disease in children in North America. Initial treatment with IVIG has significantly reduced the…
Abstract Number: 071 • 2023 Pediatric Rheumatology Symposium
Clinical Manifestations and Management of Takayasu Arteritis: A Single Center Pediatric Cohort
Ana Luiza Altaffer¹, Alvaro Orjuela² and Marietta De Guzman², ¹Texas Children's Hospital, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Takayasu arteritis (TA) is a rare granulomatous vasculitis that affects large vessels, including the aorta, its major branches, and the pulmonary artery. Data on…
Abstract Number: 031 • 2023 Pediatric Rheumatology Symposium
Multisystem Inflammatory Syndrome in Children Phenotypes Vary Between SARS-CoV-2 Variants
Greta Mastrangelo¹, Ellen Go², Paul Tsoukas², Hua Lu³, Amy Xu², Arthur Hoi Hin Cheng² and Rae Yeung⁴, ¹The Hospital of Sick Children,Department of Paediatrics, University of Toronto, Division of Rheumatology, Toronto, ON, Canada, ²The Hospital of Sick Children, Department of Paediatrics, University of Toronto, Division of Rheumatology; Cell Biology Program, The Hospital for Sick Children Research Institute, Toronto, ON, Canada, ³The Hospital for Sick Children, Toronto, ON, Canada, ⁴The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Background/Purpose: Multisystem Inflammatory Syndrome in Children (MIS-C) is a serious complication associated with COVID-19, presenting as a hyperinflammatory disorder characterized by fever and multiorgan dysfunction.…
Abstract Number: 045 • 2023 Pediatric Rheumatology Symposium
Patient Reported Outcomes in Pediatric Vasculitis
Clare Peckenpaugh¹, Aimee Hersh², CJ Inman¹, Sara Stern¹, Erin Treemarcki², Peter Merkel³ and Karen James¹, ¹University of Utah Department of Pediatrics, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT, ³University of Pennsylvania, Philidelphia, PA
Background/Purpose: Vasculitis is a group of multisystem, often relapsing diseases that can affect patients through disease activity, damage, and treatment toxicity. Vasculitis in adults often…
Abstract Number: L17 • ACR Convergence 2022
Evaluating the Safety and Factors Associated with Treatment Cessation in Takayasu Arteritis
Robert Maughan¹, Andrew Porter¹, Chanaka Dahanayake², Claudia Ianonne¹, Ritu Alapat¹, Charis Pericleous¹, Taryn Youngstein³ and Justin Mason¹, ¹Imperial College London, London, United Kingdom, ²Barts Health NHS Trust, London, United Kingdom, ³Imperial NHS Trust, London, United Kingdom
Background/Purpose: Immunosuppression in Takayasu Arteritis (TA) reduces the risk of arterial damage and disease progression. However, long-term use of glucocorticoids (GC) and other immunosuppressants carries…

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