ACR Meeting Abstracts

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  • Abstract Number: 1704 • 2019 ACR/ARP Annual Meeting

    Efficacy of Leflunomide for Treatment of Vasculitis

    NOURA MUSTAPHA 1, Lillian Barra 2, Simon Carette 3, David Cuthbertson 4, Nader A. Khalidi 5, Curry L. Koening 6, Carol Langford 7, Carol McAlear 8, Nataliya Milman 9, Larry Moreland 10, Paul Monach 11, Philip Seo 12, Ulrich Specks 13, Antoine Sreih 14, Steven Ytterberg 13, Peter Merkel 14, Christian Pagnoux15, CanVasc Canadian Vasculitis Research Network 16 and VCRC Vasculitis Clinical Research Consortium 8, 1Hopital Notre Dame, Montreal, QC, Canada, 2St Joseph's health care, London, Canada, 3Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Canada, 4University of South Florida, Tampa, FL, 5McMaster University, Hamilton, ON, Canada, 6University of Utah Hospital, Salt Lake City, UT, 7Cleveland Clinic, Cleveland, OH, 8University of Pennsylvania, Division of Rheumatology, Philadelphia, 9Arthritis Centre at the Ottawa Hospital, Riverside Campus, Ottawa, Canada, 10University of Pittsburgh, PITTSBURGH, PA, 11Brigham and Women's Hospital, Boston, MA, 12Johns Hopkins Medicine, Baltimore, MD, 13Mayo Clinic College of Medicine, Rochester, MN, 14University of Pennsylvania, Philadelphia, PA, 15Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, 16Mount Sinai Hospital, Vasculitis clinic, toronto, ON, Canada

    Background/Purpose: Only a few small case series, case reports, and one small clinical trial suggested some benefit of leflunomide (LEF) in ANCA-associated vasculitis and other…
  • Abstract Number: 1705 • 2019 ACR/ARP Annual Meeting

    Cyclophosphamide Therapy for the Neurologic Involvement of Behçet’s Disease – Is It Superior to Azathioprine in Preventing Relapses?

    Priscila Dias Cardoso Ribeiro 1, Eduarda Bonelli Zarur 1, Flávia Maria Matos Melo Campos Peixoto 1, Pedro Matos 1, Pedro Paulo de Alcantara Pedro 1, Rywka Tenenbaum Medeiros Golebiovsk 1, Livia Dutra 2 and Alexandre Wagner de Souza3, 1UNIFESP-EPM, Sao Paulo, Sao Paulo, Brazil, 2Escola Paulista de Medicina (EPM), Brazil, São Paulo, Brazil, 3UNIFESP-EPM, São Paulo, Sao Paulo, Brazil

    Background/Purpose: Neurologic involvement is one of the most serious cause of long-term morbidity and mortality in Behçet’s disease (BD). To date, no controlled trials have…
  • Abstract Number: 1706 • 2019 ACR/ARP Annual Meeting

    HLA-B*51 and Its Subtypes in Brazilian Patients with Behçet’s Disease

    Joice Belem 1, Ana Maria Fraga 2, Luís Eduardo Coelho Andrade 1 and Alexandre Wagner de Souza1, 1UNIFESP-EPM, São Paulo, Sao Paulo, Brazil, 2Fleury Group, São Paulo, Sao Paulo, Brazil

    Background/Purpose: HLA-B*51 is considered the genetic marker mostly associated with Behçet's disease (BD), mainly in countries of the silk route, where its prevalence ranges from…
  • Abstract Number: 1707 • 2019 ACR/ARP Annual Meeting

    IL-6 Promotes IgG4-related Disease by Inducing Fibroblast-dependent Tfh Cell and B Cell Differentiation Factors

    Zongfei Ji1, Rongyi Chen 2, Xiaomeng Cui 2, Lili Ma 2, Xiufang Kong 3, Lingying Ma 2, Ying Sun 2, Xiaomin Dai 2, Zhuojun Zhang 2, Huiyong Chen 2 and Lindi Jiang 2, 1Department of Rheumatology, Zhongshan hospital, Fudan University, Shanghai, China (People's Republic), 2Zhongshan hospital, Fudan University, Shanghai, China (People's Republic), 3University of Michigan & Fudan University, Ann Arbor, MI

    Background/Purpose: Considering the unsatisfied effect of conventional therapy, to investigate the pathogenesis of IgG4-related disease (IgG4-RD) is crucial to explore novel treatment strategies.  This study…
  • Abstract Number: 1708 • 2019 ACR/ARP Annual Meeting

    Azathioprine and Glucocorticoid Combination Might Be a Good Treatment Option to Achieve Remission in Patients with IgG4-related Disease

    Aydan Köken Avşar1, Onay Gercik 2, Gercek Can 3, Berrin Zengin 4, Sinem Burcu Kocaer 5, Atilla Okan Kılıç 6, Sadettin Uslu 7, Ali Karakaş 5, Merih Birlik 5, Servet Akar 8 and Fatos Onen 9, 1Dokuz Eylul University School of Medicine, Rheumatology,, Türkiye, Izmir, Turkey, 2Izmir Katip Celebi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 3Dokuz Eylul University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Izmir, Izmir, Turkey, 4Dokuz Eylul University School of Medicine, Rheumatology,, Turkey,Izmir, Izmir, Turkey, 5Division of Rheumatology, Dokuz Eylül University School of Medicine, İzmir, Turkey, Turkey,Izmir, Izmir, Turkey, 6Dokuz Eylul University School of Medicine, Internal Medıcıne, Turkey,Izmir, Izmir, Turkey, 7Division of Rheumatology, Dokuz Eylül University School of Medicine, İzmir, Turkey, Izmir, Turkey, 8Izmir Katip Celebi University, Faculty of Medicine, Division of Rheumatology, İzmir, Turkey, 9Dokuz Eylul University School of Medicine, Division of Rheumatology, İzmir, Turkey

    Background/Purpose: IgG4-related disease is a recently recognised inflammatory disease of unkown etiology, often seen in men over the age of 50 and may affect many…
  • Abstract Number: 1709 • 2019 ACR/ARP Annual Meeting

    Juvenile-Onset IgG4-Related Disease: A Systematic Review

    Eduardo Martin-Nares1, Talia Díaz-Prieto 2, Enrique Faugier-Fuentes 2, Luis A. Aparicio Vera 2 and Gabriela Hernandez-Molina 1, 1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 2Department of Rheumatology, Hospital Infantil de México Federico Gómez, Mexico City, Mexico

    Background/Purpose: IgG4-related disease (IgG4-RD) presenting in the pediatric age is rare. Natural history and outcomes are not well defined. The aim of this study was…
  • Abstract Number: 1710 • 2019 ACR/ARP Annual Meeting

    Chemokine and Cytokine Tear Profile of Patients with IgG4-Related Disease

    Eduardo Martin-Nares1, Luis Llorente 1, Guadalupe Lima 1, Diego Hernández-Ramírez 1, Isela Chan-Campos 1, Vanessa Saavedra-González 1 and Gabriela Hernandez-Molina 1, 1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Background/Purpose: The lacrimal gland is frequently involved in both IgG4-related disease (IgG4-RD) and Sjögren’s syndrome (SS) and presents with swelling and/or dry eye symptoms. Although…
  • Abstract Number: 1711 • 2019 ACR/ARP Annual Meeting

    Distinctive Imaging Features Between IgG4-Related Ophthalmic Disease and Graves’ Orbitopathy: A Comparative Study

    Eduardo Martin-Nares1, Oziel Amaya-Piña 2, Rosa Delia Delgado Hernández 2 and Gabriela Hernandez-Molina 1, 1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 2Department of Radiology and Molecular Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Background/Purpose: IgG4-related ophthalmic disease (IgG4-ROD) may present as a cause of orbital myositis leading to proptosis and diplopia. This clinical scenario can be mistakenly diagnosed…
  • Abstract Number: 1712 • 2019 ACR/ARP Annual Meeting

    Head and Neck Involvement of IgA Vasculitis: A Case-Control Study

    Michel Villatoro-Villar1, David Wetter 1, Cynthia Crowson 2, Kenneth Warrington 1 and Matthew Koster 1, 1Mayo Clinic Rochester, Rochester, MN, 2Mayo Clinic Rochester, Rochester

    Background/Purpose: IgA vasculitis (IgAV) is an immune-complex mediated, small-vessel vasculitis which predominantly involves the skin on the lower extremities. Head and neck involvement is rarely…
  • Abstract Number: 1713 • 2019 ACR/ARP Annual Meeting

    Drug-induced IgA Vasculitis: Data from the French Pharmacovigilance Network and the WHO VigiBase

    CAMILLE RASMUSSEN1, MYLENE TISSEYRE 2, JULIE GARON-CZMIL 2, MARINA ATZENHOFFER 3, Loïc Guillevin 4, LAURENT CHOUCHANA 2 and Benjamin Terrier 4, 1Cochin Hospital, National Referral Center for Rare Systemic Autoimmune Diseases, PARIS, PARIS, France, 2PHARMACOVIGILANCE, COCHIN HOSPITAL, PARIS, PARIS, France, 3PHARMACOVIGILANCE CENTER LYON, FRANCE, LYON, France, 4National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

    Background/Purpose: IgA vasculitis (IgAV) is an immune complex small-vessel vasculitis, with IgA1-dominant immune deposits. Drug-induced IgAV were rarely reported in the literature, but no systematic…
  • Abstract Number: 1714 • 2019 ACR/ARP Annual Meeting

    Can We Predict Early Relapses in Adult IgA Vasculitis?

    Jaka Ostrovrsnik1, Ziga Rotar 1, Matija Tomsic 1 and Alojzija Hocevar 1, 1Department of Rheumatology, University Medical Center Ljubljana, Slovenia, Ljubljana, Slovenia

    Background/Purpose: In adults, IgA vasculitis (IgAV) frequently takes a severe course in the acute phase of the disease. Data on long term outcomes is limited.…
  • Abstract Number: 1715 • 2019 ACR/ARP Annual Meeting

    Predictors of Gastrointestinal and Renal Involvement in Adult IgA Vasculitis

    Alojzija Hocevar1, Ziga Rotar 2 and Matija Tomšič 3, 1Department of Rheumatology, University Medical Center Ljubljana, Slovenia, LJUBLJANA, Slovenia, 2UMC LJUBLJANA, DPT. OF RHEUMATOLOGY, LJUBLJANA, Slovenia, 3Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, LJUBLJANA, Slovenia

    Background/Purpose: IgA vasculitis (IgAV) is a common vasculitis of adult population, yet the disease in adults is still poorly defined. The aim of our study…
  • Abstract Number: 1716 • 2019 ACR/ARP Annual Meeting

    Poor-Prognosis Factors of Systemic Vasculitides with Gastrointestinal Involvement: Data from a Large Retrospective Study

    Segolene Gendreau1, Benjamin Thoreau 2, François Maurier 3, Tiphaine Goulenok 1, Laure Frumholtz 1, Arsène Mékinian 4, Saskia Oro 5, Charlotte Bernigaud 5, Alexandra Audemard 1, Maxime Samson 6, Antoine Gaillet 1, Romain Sonneville 1, Jean-Benoit Arlet 1, Adrien Mirouse 1, Jean-Emmanuel Kahn 7, Aurélie Hummel 1, Thomas Pires 8, Wendy Jourde 8, Eric Hachulla 9, Pierre-Louis Carron 10, Cécile-Audrey Durel 11, Francoise Sarrot-Reynauld 12, Nathalie Tieulie 13, Elisabeth Diot 14, Christian Agard 15, Guillaume Armengol 16, Alice Berezne 17, Christophe Deligny 18, Fleur Cohen 1, Stanislas Faguer 19, Guillaume Le Guenno 20, Mohamed Hamidou 15, Loïc Guillevin 21 and Benjamin Terrier 21, 1APHP, Paris, France, 2CHU Tours, Tours, France, 3Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, 4Hôpital Saint-Antoine, APHP, Paris, Paris, France, 5APHP, Creteil, France, 6Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, 7APHP, Boulogne Billancourt, France, 8CHU Bordeaux, Bordeaux, France, 9CHU Lille, Lille, France, 10Grenoble, Grenoble, France, 11Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France, Lyon, France, 12CHU Grenoble, Grenoble, France, 13University Hospital of Nice, Nice, France, 14APHP, Tours, France, 15CHU Nantes, Nantes, France, 16CHU Rouen, Rouen, France, 17CH Annecy Genevois, Annecy, France, 18CHU Martinique, Fort de France, France, 19CHU Toulouse, Toulouse, France, 20CHU Clermont Ferrand, Clermont Ferrand, France, 21National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France

    Background/Purpose: Gastrointestinal (GI) involvement was described as a poor-prognosis factor of systemic necrotizing vasculitides. However, the prognostic impact of GI involvement may vary according to…
  • Abstract Number: 1717 • 2019 ACR/ARP Annual Meeting

    Identifying Patterns of Histopathologic Presentation in CNS Vasculitis

    Chan Mi Lee1, Richard Prayson 2, Marcia Leon Rabanal 3, Leonard Calabrese 3 and Rula Hajj-ali 3, 1Cleveland Clinic Lerner College of Medicine of Case Western Reserve University/Cleveland Clinic, Cleveland, OH, 2Anatomic Pathology/Cleveland Clinic, Cleveland, OH, 3Rheumatologic and Immunologic Disease/Cleveland Clinic, Cleveland, OH

    Background/Purpose: Primary central nervous system vasculitis (CNS-V) is vasculitis confined to the brain, spinal cord and meninges. It is a rare condition with unknown pathogenesis.…
  • Abstract Number: 1718 • 2019 ACR/ARP Annual Meeting

    Geographic Disparities in Mortality Rates of Vasculitis in the United States: 1999 to 2017

    Alicia Rodriguez-Pla1, 1University of Arizona/Banner Health Medical Center, Tucson, AZ

    Background/Purpose: Earlier diagnosis and less toxic immunosuppressive therapies have improved the survival of patients with vasculitis. Current data on mortality rates of vasculitis are limited.…
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