ACR Meeting Abstracts

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  • Abstract Number: 937 • 2017 ACR/ARHP Annual Meeting

    Treatment Response in Polyarticular JIA Is Associated with Transcriptional Changes and Chromatin Reorganization in CD4+ T Cells

    Evan Tarbell1, Kaiyu Jiang2, Yanmin Chen2, Tao Liu3 and James Jarvis4, 1Biochemistry, University at Buffalo, Buffalo, NY, 2Pediatrics, University at Buffalo, Buffalo, NY, 3Biochemistry, University at Buffalo Jacobs School of Medicine, Buffalo, NY, 4Department of Genetics, Genomics & Bioinformatics, University at Buffalo, Buffalo, NY

    Background/Purpose: To identify transcriptional changes in CD4+ T cells as children with polyarticular JIA transition from active disease to remission, and to identify underlying changes…
  • Abstract Number: 938 • 2017 ACR/ARHP Annual Meeting

    A Germline Macrophage Activation Syndrome-Associated Nlrc4 Mutation Causes Chronic, Systemic, Non-Hematopoietic IL-18 Elevation and Intestinal MHC-II Upregulation

    Eric Weiss1, Corinne Schneider2 and Scott Canna3, 1RK Mellon Institute, Children’s Hospital of Pittsburgh, Pittsburgh, PA, 2Pediatrics, Children's Hospital Pittsburgh, Pittsburgh, PA, 3NIAMS, National Institutes of Health, Bethesda, MD

    Background/Purpose: Patients prone to the development of Macrophage Activation Syndrome (MAS) can have extreme and often chronic elevation in the pro-inflammatory cytokine interleukin-18 (IL-18). In…
  • Abstract Number: 939 • 2017 ACR/ARHP Annual Meeting

    Stimulator of Interferon Genes (STING)-Induced Endothelial-Mesenchymal Transition (EndMT) Contributes to Interstitial Lung Disease in Sting-Associated Vasculopathy with Onset in Infancy (SAVI) Patients

    Louise Malle1, Dan Yang2, Adriana Almeida de Jesus1, Guibin Chen2, Bernadette Marrero1, Gina A. Montealegre Sanchez1, Yin Liu3, Gregor Dueckers4, Suzanne Ramsey5, Joseph Fontana6, Rachel VanTries1, Yan Huang1, Laisa Santiago7, Benito Gonzalez8, Paul Brogan9, Juergen Brunner10, Ebun Omoyinmi11, Athimalaipet V. Ramanan12, Amy Paller13, Olcay Y. Jones14, Seza Ozen15, Stephen R. Brooks16, Manfred Boehm17 and Raphaela Goldbach-Mansky1, 1Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, 2Center for Molecular Medicine, NHLBI/NIH, Bethesda, MD, 3Scientific Review Branch, NIAMS/NIH, Bethesda, MD, 4Helios Kliniken - Kinderklinik, HELIOS Klinikum Krefeld, Krefeld, Germany, 5Pediatric Rheumatology, IWK Health Centre, Dalhousie University, Halifax, NS, Canada, 6Cardiovascular and Pulmonary Branch, NHLBI/NIH, Bethesda, MD, 7Johns Hopkins All Children's Hospital Rheumatology, Saint Petersburg, FL, 8Luis Calvo Mackenna Hospital, Santiago, Chile, 9Infection Inflammation and Rheumatology, UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 10Division of Pediatric Rheumatology, Medical University Innsbruck, Innsbruck, Austria, 11University College London Institute of Child Health, London, United Kingdom, 12Bristol Royal Hospital for Children, Bristol, United Kingdom, 13Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IN, 14Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, MD, 15Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 16Biodata Mining and Discovery Section, Office of Science and Technology, NIAMS/NIH, Bethesda, MD, 17Center for Molecular Medicine, NHLBI/ NIH, Bethesda, MD

    Background/Purpose: Pulmonary fibrosis, is a life-threatening complication of the monogenic autoinflammatory interferonopathy, STING-Associated Vasculopathy with onset in Infancy (SAVI) that is caused by gain-of-function mutations…
  • Abstract Number: 940 • 2017 ACR/ARHP Annual Meeting

    IL1RN Variation Is Associated with Systemic Juvenile Idiopathic Arthritis and Predicts Non-Response to Anakinra Treatment

    Emily Shuldiner1, Victoria Arthur1, Anne Hinks2, Patricia Woo3, Wendy Thomson2, Elaine F. Remmers4 and Michael J. Ombrello1, 1Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, 2Arthritis Research UK Centre for Genetics and Genomics, Centre for Musculoskeletal Research, Manchester Academic Health Sciences Centre, The University of Manchester, Manchester, United Kingdom, 3University College London, London, United Kingdom, 4Genetics and Genomics Branch, NIH, NIAMS, Bethesda, MD

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood inflammatory disease whose pathophysiology is poorly understood. sJIA is phenotypically heterogeneous with variable manifestations and responses…
  • Abstract Number: 941 • 2017 ACR/ARHP Annual Meeting

    Ro/SSA Autoantibody Exposed Neonates Have an Expansion of NK Cells and a Discernible Type II IFN Signature with High IFNγ in Peripheral Blood

    Margarita Ivanchenko1, Malin Hedlund1, Gudny Ella Thorlacius1, Vijole Ottosson1, Karine Chemin2, Sven-Erik Sonesson3 and Marie Wahren-Herlenius1, 1Unit of Experimental Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, 2Department of Medicine, Center for Molecular Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, 3Pediatric Cardiology Unit, Department of Women´s and Children´s Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden

    Background/Purpose: Congenital heart block (CHB) may develop in the fetus of women with Ro/SSA autoantibodies. The mothers are commonly diagnosed with Sjögren’s syndrome or SLE.…
  • Abstract Number: 942 • 2017 ACR/ARHP Annual Meeting

    Role of the Pyrin Inflammasome in Resistance to Yersinia Pestis: A Possible Selective Advantage for Carriers of MEFV Mutations

    Yong Hwan Park1, Wonyong Lee1, Lawton Chung2, James Bliska2, Daniel L. Kastner1 and Jae Jin Chae3, 1Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 2Department of Molecular Genetics and Microbiology, School of Medicine, Stony Brook University, Stony Brook, NY, 3Inflammatory disease section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

    Background/Purpose: Mutations in MEFV, encoding pyrin, cause the prototypic autoinflammatory disease, familial Mediterranean fever (FMF). The carrier frequency of FMF-associated MEFV mutations is extraordinarily high…
  • Abstract Number: 943 • 2017 ACR/ARHP Annual Meeting

    The Course of the Forced Vital Capacity during Treatment for Systemic Sclerosis-Related Interstitial Lung Disease Predicts Long-Term Survival in 2 Independent Cohorts

    Elizabeth R. Volkmann1, Donald P. Tashkin1, Myung Sim1, Dinesh Khanna2, Michael Roth3, Philip J. Clements3, Daniel E. Furst1, Lynette Keyes-Elstein4, Ashley Pinckney4, Ellen Goldmuntz5, Robert Elashoff6 and Keith Sullivan7, 1University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 2University of Michigan, Ann Arbor, MI, 3Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 4Rho Federal Systems, Inc., Chapel Hill, NC, 5NIAID, NIH, Bethesda, MD, 6University of California, Los Angeles, Los Angeles, CA, 7Duke University, Durham, NC

    Background/Purpose: While prior observational studies have identified predictors of mortality in systemic sclerosis-interstitial lung disease (SSc-ILD), no studies have evaluated predictors of long-term mortality in…
  • Abstract Number: 944 • 2017 ACR/ARHP Annual Meeting

    Long-Term Survival and Follow-up of Anti-Th/to Antibody Positive Systemic Sclerosis Patients

    Devon Charlton1, Maureen Laffoon2, Thomas A. Medsger Jr.3 and Robyn T. Domsic4, 1Division of Rheumatology and Clinical Immunology, University of Pittsburgh Medical Center, Pittsburgh, PA, 2Rheumatology, University of Pittsburgh Medical Center, Pittsburgh, PA, 3Department of Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, 4Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Anti-Th/To antibody is an autoantibody associated with systemic sclerosis (SSc), occurring in 5-10% of patients. To date, only relatively small case series have described…
  • Abstract Number: 945 • 2017 ACR/ARHP Annual Meeting

    Autoantibodies to the hPOP1 and Rpp25/38 Components of the Th/to Complex Identify a Subgroup of Systemic Sclerosis (SSc) Associated Interstitial Lung Disease (ILD) and Antibodies to hPOP1 Are Associated with Reduced Survival

    Jennifer G Walker1, Mandana Nikpour2, Molla Huq3, Karen Patterson1, Peter Roberts-Thomson4, Susanna Proudman5, Wendy Stevens6, Susan Lester7, Maureen Rischmueller8, Jane Zochling9, Joanne Sahhar10, Peter Nash11, Janet Roddy12, Catherine Hill13, Marie Hudson14, Murray Baron15, Janet E. Pope16, Maureen D. Mayes17, Shervin Assassi18, Michael Mahler19 and Marvin J. Fritzler20, 1Flinders University of South Australia, Adelaide, Australia, 2Melbourne University, Melbourne, Australia, 3Department of Medicine (Rheumatology), Melbourne University, Melbourne, Australia, 4Immunology, Flinders University of South Australia, Adelaide, Australia, 5University of Adelaide, Adelaide, Australia, 6Rheumatology, St. Vincent’s Hospital, Melbourne, Australia, 7Queen Elizabeth Hospital, Adelaide, Australia, 8Medicine, University of Adelaide, Adelaide, Australia, 9Menzies Institute for Medical Research, Tasmania, Hobart, Australia, 10Department of Rheumatology, Monash Medical Centre, Melbourne, Australia, 11University of Queensland, Brisbane, Australia, 12Royal Perth Hospital, Perth, Australia, 13Medicine, The University of Adelaide, Adelaide, Australia, 14Division of Rheumatology, Jewish General Hospital, Lady David Institute for Medical Research, Montreal, QC, Canada, 15Medicine, McGill University, Quebec, Montreal, QC, Canada, 16Department of Medicine, Division of Rheumatology, University of Western Ontario, St Joseph's Health Care, London, ON, Canada, 17Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 18University of Texas McGovern Medical School, Houston, TX, 19Research and Development, Inova Diagnostics, San Diego, CA, 20Medicine, University of Calgary, Calgary, AB, Canada

    Background/Purpose: The clinical associations of anti-Th/To antibodies (Abs) are not fully established, and until recently immunoprecipitation (IP) was the only reliable assay. Using IP, anti-Th/To…
  • Abstract Number: 946 • 2017 ACR/ARHP Annual Meeting

    Clinical and Serological Features of Systemic Sclerosis in a Multicenter African American Cohort: Analysis of the Genome Research in African American Scleroderma Patients Clinical Database

    Nadia D. Morgan1, Ami A. Shah1, Maureen D. Mayes2, Robyn T. Domsic3, Thomas A. Medsger Jr.4, Virginia D. Steen5, John Varga6, Mary A. Carns7, Paula S. Ramos8, Richard M. Silver9, Elena Schiopu10, Dinesh Khanna10, Vivien Hsu11, Jessica K. Gordon12, Heather Gladue13, Lesley A. Saketkoo14, Lindsey A. Criswell15, Chris T. Derk16, Marcin A. Trojanowski17, Victoria K. Shanmugam18, Lorinda Chung19, Antonia Valenzuela20, Reem Jan21, Avram Goldberg22, Elaine F. Remmers23, Daniel L. Kastner23, Fredrick M. Wigley24, Pravitt Gourh25 and Francesco Boin26, 1Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 2Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 3Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, 4Department of Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, 5Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, 6Rheumatology and Dermatology, Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 7Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 8Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, 9Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, 10University of Michigan, Ann Arbor, MI, 11Rheumatology, Robert Wood Johnson University Scleroderma Program, New Brunswick, NJ, 12Rheumatology, Hospital for Special Surgery, New York, NY, 13Rheumatology, Arthritis and Osteoporosis Consultants of the Carolinas, Charlotte, NC, 14Rheumatology, Tulane University School of Medicine, New Orleans, LA, 15Medicine/Rheumatology, University of California, San Francisco, San Francisco, CA, 16Rheumatology, University of Pennsylvania, Philadelphia, PA, 17Boston University School of Medicine, Boston, MA, 18Rheumatology, The George Washington University, Washington, DC, 19Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, 20Stanford University School of Medicine, Stanford, CA, 21Medicine, Rheumatology, University of Chicago, Chicago, IL, 22NYU Langone Medical Center, New York, NY, 23National Institutes of Health (NIH), National Human Genome Research Institute, Bethesda, MD, 24Rheum Div/Mason F Lord, Johns Hopkins University, Baltimore, MD, 25NIAMS-Rheumatology, National Institutes of Health (NIH), National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, 26Rheumatology, University of California, San Francisco, San Francisco, CA

    Background/Purpose: Racial differences exist in the severity of systemic sclerosis (SSc). To enhance our knowledge about SSc in African Americans, we established a comprehensive clinical…
  • Abstract Number: 947 • 2017 ACR/ARHP Annual Meeting

    Norway As a National Reference Population for Systemic Sclerosis; Preliminary Results from a Complete, Nationwide Cohort

    Anna-Maria Hoffmann-Vold1, Håvard Fretheim1, Anne Kristine Halse2, Marit Seip3, Marianne Wallenius4, Helle Bitter5, Torhild Garen1, Oyvind Midtvedt1 and Øyvind Molberg1, 1Oslo University Hospital, Oslo, Norway, 2Haukeland University Hospital, Bergen, Norway, 3University Hospital of North Norway, Tromso, Norway, 4St. Olav's University Hospital, Trondheim, Norway, 5Hospital of Southern Norway, Kristiansand, Norway

    Background/Purpose: To fully understand the impact of Systemic sclerosis (SSc) there is a need to complement existing multi-center registry data with novel, unbiased, high resolution…
  • Abstract Number: 948 • 2017 ACR/ARHP Annual Meeting

    Application of a Diagnostic Algorithm to Identify Inflammatory Myopathy in Systemic Sclerosis

    Vandana Bhushan1,2, Adam Maundrell1, Charlotte Proudman1,2, Leah McWilliams1, Llew Spargo1, Robert Metcalf1, Jennifer Walker3, Mandana Nikpour4,5, Wendy Stevens4, Vidya Limaye1,2 and Susanna Proudman1,2, 1Rheumatology Unit, Royal Adelaide Hospital, South Australia, Adelaide, Australia, 2Discipline of Medicine, University of Adelaide, South Australia, Adelaide, Australia, 3Flinders University of South Australia, Adelaide, Australia, 4St Vincent's Hospital, Melbourne, Victoria, Melbourne, Australia, 5Department of Medicine, University of Melbourne, Victoria, Melbourne, Australia

    Background/Purpose: Muscle involvement in systemic sclerosis (SSc) is under-recognised and poorly understood. Reported prevalence varies up to 15%, reflecting lack of consistent definition, the heterogeneous…
  • Abstract Number: 949 • 2017 ACR/ARHP Annual Meeting

    Sequence Homology and Immune Reactivity between T Cell Epitopes of Related Gut Microbes and Two Novel Autoantigens Provide a Link between Microbial and Host Immunity in Patients with Rheumatoid Arthritis

    Annalisa Pianta1, Sheila Arvikar2, Klemen Strle3, Elise E. Drouin1, Qi Wang4, Catherine E. Costello4 and Allen C. Steere5, 1Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 2Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, BOSTON, MA, 3Department of Immunology and Inflammatory Diseases, Massachusetts General Hospital, BOSTON, MA, 4Center for Biomedical Mass Spectrometry, Boston University School of Medicine, Boston, MA, 5Center for Immunolgy and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, MA

    Background/Purpose:  It has been proposed that immunological triggers at mucosal sites, such as the gut microbiota, may promote autoimmunity affecting joints in patients with rheumatoid…
  • Abstract Number: 950 • 2017 ACR/ARHP Annual Meeting

    Identification of Naturally Processed Immunodominant Topoisomerase I Epitopes in Patients with Systemic Sclerosis

    Eleni Tiniakou1, Andrea Fava2, Tara Guhr3, Francesco Boin4 and Erika Darrah5, 1Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 2Johns Hopkins University, Baltimore, MD, 3University of North Carolina, Chapel Hill, NC, 4Rheumatology, University California, San Francisco, San Francisco, CA, 5Department of Medicine/Division of Rheumatology, The Johns Hopkins University School of Medicine, Baltimore, MD

    Background/Purpose: Identification of immunodominant T cell epitopes of autoantigens is crucial to understanding the pathogenesis of autoimmune diseases and developing disease-specific diagnostic and therapeutic tools.…
  • Abstract Number: 951 • 2017 ACR/ARHP Annual Meeting

    Cross Sectional Analysis of Citrullinated-Synovial Antigen-Specific CD4+ T Cells in an RA Cohort Demonstrates Antigen Based Differences in T Cell Frequency, Phenotype and the Influence of Immunotherapy

    Cliff Rims1, Sylvia Posso1, Bernard Ng2, Jeffrey Carlin3, Eddie James4 and Jane H. Buckner4, 1Translational Research, Benaroya Research Institute at Virginia Mason, Seattle, WA, 2Rheumatology, VA Puget Sound Healthcare System, Seattle, WA, 3Rheumatology, Virginia Mason Medical Center, Seattle, WA, 4Benaroya Research Institute at Virginia Mason, Seattle, WA

    Background/Purpose: The presence of ACPA in RA indicates that an immune response directed toward citrullinated synovial antigens participates in disease development or persistence. Research from…
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