ACR Meeting Abstracts

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  • Abstract Number: 804 • 2019 ACR/ARP Annual Meeting

    STING-associated Vasculopathy with Onset in Infancy (SAVI Syndrome) Can Mimic Juvenile Idiopathic Arthritis

    Jose Luis Tandaipan1, Mireia Lopez Corbeto 2 and Estefania Moreno Ruzafa 3, 1Hospital Universitari Mutua Terrassa, Terrassa, Spain, 2Hospital Universitari Vall d'Hebron, Barcelona, Catalonia, Spain, 3Hospital Universitari Vall d'Hebron, Barcelona, Spain

    Background/Purpose: Gain-of-function mutations in TMEM173 encoding STING (Stimulator of Interferon Genes) underlie a novel type I INF termed SAVI syndrome. It is characterized by a…
  • Abstract Number: 805 • 2019 ACR/ARP Annual Meeting

    Risk of Cardiovascular Disease in SLE Is Significant Early and Highlights Racial Disparities

    Shivani Garg1, Christie Bartels 1, Cristina Drenkard 2, Gaobin Bao 2 and S Sam Lim 2, 1University of Wisconsin School of Medicine and Public Health, Madison, WI, 2Emory University, Atlanta, GA

    Background/Purpose: SLE is an independent risk factor for premature cardiovascular disease (CVD) and afflicts African Americans (AA’s) 3 times more than whites. Two predominantly white…
  • Abstract Number: 806 • 2019 ACR/ARP Annual Meeting

    A Randomized, Controlled Trial of Rituximab versus Azathioprine After Induction of Remission with Rituximab for Patients with ANCA-associated Vasculitis and Relapsing Disease

    Rona Smith1, David Jayne 2 and Peter Merkel 3, 1University of Cambridge, Cambridge, England, United Kingdom, 2Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, 3University of Pennsylvania, Philadelphia

    Background/Purpose: Rituximab is an effective therapy for induction of remission in ANCA-associated vasculitis (AAV).  However, the effect of rituximab is not sustained, and relapse rates…
  • Abstract Number: 807 • 2019 ACR/ARP Annual Meeting

    Guselkumab, an Anti-interleukin-23p19 Monoclonal Antibody, in Patients with Active Psoriatic Arthritis Who Were Biologic-Naïve or Prior TNFα Inhibitor-Treated: Week 24 Results of a Phase 3, Randomized, Double-blind, Placebo-controlled Study

    Atul Deodhar1, Philip Helliwell 2, Wolf-Henning Boencke 3, Elizabeth Hsia 4, Alexa Kollmeier 5, Ramanand Subramanian 5, Xie Xu 5, Shihong Sheng 5, Bei Zhou 5 and Christopher Ritchlin 6, 1Oregon Health & Science University, Portland, OR, 2University of Leeds, Leeds, United Kingdom, 3Geneva Univ Hospitals, Geneva, Switzerland, 4Janssen Research & Development, LLC/University of Pennsylvania, Spring House/Philadelphia, PA, 5Janssen Research & Development, LLC, Spring House, PA, 6Division of Allergy, Immunology and Rheumatology, Center for Musculoskeletal Research, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA, Rochester, NY

    Background/Purpose: Guselkumab (GUS), an anti-interleukin-23p19 monoclonal antibody, is approved to treat PsO. We evaluated GUS efficacy and safety in a Phase 3, double-blind, PBO-controlled trial…
  • Abstract Number: 808 • 2019 ACR/ARP Annual Meeting

    Long-Term Outcome of Tocilizumab for Patients with Giant Cell Arteritis: Results from Part 2 of a Randomized Controlled Phase 3 Trial

    John Stone1, Min Bao 2, Jian Han 2, Martin Aringer 3, Daniel Blockmans 4, Elisabeth Brouwer 5, Maria C. Cid 6, Bhaskar Dasgupta 7, Jürgen Rech 8, Carlo Salvarani 9, Robert Spiera 10 and Sebastian Unizony 1, 1Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, 2Genentech, South San Francisco, CA, 3Division of Rheumatology, Department of Medicine III, University Medical Center & Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany, Dresden, Germany, 4Department of General Internal Medicine, University Hospitals Gasthuisberg, Leuven, Belgium, 5Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 6Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain, 7Southend University Hospital, NHS Foundation Trust, Westcliff-on-Sea, United Kingdom, 8Department of Internal Medicine 3, Friedrich-Alexander-University Erlangen-Nuremberg (FAU) and Universitätsklinikum Erlangen, Erlangen, Germany, 9Division of Rheumatology, Arcispedale Santa Maria Nuova-IRCCS, Reggio Emilia, Italy, 10Hospital for Special Surgery, New York, NY

    Background/Purpose: Tocilizumab (TCZ) 162 mg administered subcutaneously weekly (QW) or every-other-week (Q2W) plus 26-wk prednisone tapering resulted in higher rates of sustained glucocorticoid (GC)–free remission…
  • Abstract Number: 809 • 2019 ACR/ARP Annual Meeting

    TNF-α Drives Progressive Obliterative Pulmonary Vascular Disease and Represents a Novel Model of Connective-Tissue Disease Associated Pulmonary Arterial Hypertension (CTD-PAH)

    Benjamin Korman1, Richard Bell 1, R James White 2, Maria de la luz Garcia-Hernandez 3, Emily Wu 2, Pamela Slattery 2, Nelson Huertas 2, Stacey Duemmel 2, Marc Nuzzo 2, Homaira Rahimi 4, Craig Morrell 2, Christopher Ritchlin 3 and Edward Schwarz 2, 1University of Rochester Medical Center, Rochester, NY, 2University of Rochester, Rochester, NY, 3Division of Allergy, Immunology and Rheumatology, Center for Musculoskeletal Research, University of Rochester School of Medicine and Dentistry, Rochester, NY, USA, Rochester, NY, 4University of Rochester Medical Center, Rochester

    Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe cardiopulmonary disease characterized by an obliterative vasculopathy and vascular remodeling, right heart hypertrophy, and premature death.  Connective…
  • Abstract Number: 810 • 2019 ACR/ARP Annual Meeting

    Small Vessel Vasculitis Syndrome with Autoinflammation Caused by De Novo Mutations in LYN Kinase

    Adriana de Jesus1, Gina Montealegre Sanchez 2, Helen Freeman 3, Neil Martin 4, Ebun Omoyinmi 5, Katherine Calvo 6, Richard Chyi-chia Lee 7, Murray Passo 8, Natasha Ruth 8, David Kleiner 7, Yan Huang 9, Nirali Shah 10, Paul Brogan 11, SuJin Hwang 12, HyeSun Kuehn 12, Sergio Rosenzweig 12, Zuoming Deng 13, Anna Huttenlocher 14, Susan Moir 15, Douglas Kuhns 16 and Raphaela Goldbach-Mansky 17, 1Translation Autoinflammatory Diseases Section/NIAID/NIH, Silver Spring, MD, 2Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, 3Raigmore Hospital, Inverness, United Kingdom, 4Royal Hospital for Children, Glasgow, United Kingdom, 5University College London Institute of Child Health, London, United Kingdom, 6Hematology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, 7Laboratory of Pathology/NCI/NIH, Bethesda, MD, 8Medical University of South Carolina, Charleston, SC, 9Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, MD, 10Pediatric Oncology Branch/NCI/NIH, Bethesda, MD, 11Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 12Immunology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, 13Biomining and Discovery Section/NIAMS/NIH, Bethesda, MD, 14Department of Pediatrics/University of Wisconsin, Madison, WI, 15Immunopathogenesis Section/NIAID/NIH, Bethesda, MD, 16Collaborative Clinical Research Branch/NIAID/NIH, Bethesda, MD, 17Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD

    Background/Purpose: Lyn kinase is a member of the Src family of non-receptor tyrosine-protein kinases that modifies signals from various cell surface receptors and regulates innate…
  • Abstract Number: 811 • 2019 ACR/ARP Annual Meeting

    Monomethyl Fumarate as a Novel Therapy for Macrophage Activation Syndrome: Mechanism of Action in an Animal Model

    Chhanda Biswas 1, Thomas Burn 1, Niansheng Chu 1 and Edward Behrens1, 1The Children's Hospital of Philadelphia, Philadelphia

    Background/Purpose: Macrophage activation syndrome (MAS) is a deadly systemic inflammatory condition marked by an increase in enzymes of iron metabolism including ferritin and heme-oxygenase 1…
  • Abstract Number: 812 • 2019 ACR/ARP Annual Meeting

    Sex Differences in Autoimmunity and Cardiovascular Risk Could Be Associated with Altered Treg Phenotype and Lipoprotein Metabolism

    George Robinson1, Kirsty Waddington 1, Anna Radziszewska 1, Hannah Peckham 1, David A Isenberg 2, Yiannis Ioannou 1, Coziana Ciurtin 3, Ines Pineda-Torra 1 and Elizabeth Jury 1, 1University College London, London, United Kingdom, 2Centre for Rheumatology, London, United Kingdom, 3Centre for Adolescent Rheumatology, University College London London, UK, Londond, United Kingdom

    Background/Purpose: Males and females have altered immune responses resulting in variation in autoimmune risk. Sex differences exist in the frequency and activity of immune-cell subsets…
  • Abstract Number: 813 • 2019 ACR/ARP Annual Meeting

    Monocyte and Macrophage Transcriptional Phenotypes in Systemic Juvenile Idiopathic Arthritis Reveal TRIM8 as a Mediator of IFNγ Hyperresponsiveness and Risk for Macrophage Activation Syndrome

    Grant Schulert1, Thuy Do 1, Sanjeev Dhakal 1, Ndate Fall 2, Mario Medvedovich 3, Sherry Thornton 1, Nathan salomonis 2 and Alexei A. Grom 1, 1Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 3University of Cincinnati College of Medicine, Cincinnati, OH

    Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe and distinct subtype of childhood arthritis. Children with SJIA are at risk for macrophage activation syndrome…
  • Abstract Number: 814 • 2019 ACR/ARP Annual Meeting

    Interferon Signature and Cytokine Patterns Define Novel Autoinflammatory Diseases

    Adriana de Jesus1, Yangfeng Hou 2, Stephen Brooks 3, Louise Malle 4, Angelique Biancotto 5, Yan Huang 6, Katherine Calvo 7, Bernadette Marrero 8, Susan Moir 9, Andrew Oler 10, Zuoming Deng 3, Gina Montealegre Sanchez 1, Scott Canna 11 and Raphaela Goldbach-Mansky 12, 1Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, 2Department of Rheumatology, Shandong Provincial Qianfoshan Hospital, Shandong University, Shandong, China (People's Republic), 3Biomining and Discovery Section/NIAMS/NIH, Bethesda, MD, 4Translational Autoinflammatory Diseases Section (TADS), NIAID/NIH, Bethesda, 5NIH/CHI, Bethesda, 6Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, MD, 7Hematology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, 8Computational Systems Biology Section/NIAID/NIH, Bethesda, 9Immunopathogenesis Section/NIAID/NIH, Bethesda, MD, 10NIH/NIAID, Bethesda, 11UPMC Children's Hospital of Pittsburgh, Pittsburgh, 12Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD

    Background/Purpose: Undifferentiated systemic autoinflammatory diseases (USAID) present diagnostic and therapeutic challenges.  Cytokine dysregulation may identify disease groups that respond to targeted treatments. IFN-signaling blocking treatments…
  • Abstract Number: 815 • 2019 ACR/ARP Annual Meeting

    Expression Profiling of Genes in Rheumatoid Fibroblast‑like Synoviocytes Regulated by Fas Ligand Using cDNA Microarray Analysis

    Koji Fukuda1, Yasushi Miura 2, Shinya Hayashi 1, Toshihisa Maeda 3 and Ryosuke Kuroda 1, 1Department of Orthopaedic Surgery, Kobe University Graduate School of Medicine, Kobe, Japan, 2Division of Orthopedic Science, Department Of Rehabilitation Science, Kobe University Graduate School of Health Sciences, Kobe, Japan, 3Matsubara Mayflower Hospital, Kobe, Japan

    Background/Purpose: Fas ligand (FasL) is a member of tumor necrosis factor superfamily (TNFSF6) and reported to contribute to synovial hyperplasia of rheumatoid arthritis (RA). Apoptosis…
  • Abstract Number: 816 • 2019 ACR/ARP Annual Meeting

    The Long Non-coding RNA HOTAIR Regulates BMP2 and Wnt Pathways in Synovial Fibroblasts

    Muriel Elhai1, Raphael Micheroli 1, Mojca Frank-Bertoncelj 2, Kerstin Klein 1, Oliver Distler 3 and Caroline Ospelt 2, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland., Zürich, Switzerland, 2University Hospital Zürich, Zürich, Switzerland, 3Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland

    Background/Purpose: Mechanisms involved in joint patterning of rheumatic diseases remain unknown. The long non-coding RNA HOTAIR is exclusively expressed in synovial fibroblasts (SF) from lower…
  • Abstract Number: 817 • 2019 ACR/ARP Annual Meeting

    Enhanced Expression of mRNA for TAK1 in CD34+ Cells of the Bone Marrow in Rheumatoid Arthritis

    Shunsei Hirohata1, Tatsuo Nagai 2, Tetsuya Tomita 3 and Hideki Yoshikawa 3, 1Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine, Tatsuno, Japan, 2Kitasato University School of Medicine, Sagamihara, Japan, 3Osaka University Graduate School of Medicine, Osaka, Japan

    Background/Purpose: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial hyperplasia, consisting of type A macrophage-like synoviocytes and type B fibroblast-like synoviocytes (FLS).…
  • Abstract Number: 818 • 2019 ACR/ARP Annual Meeting

    Semaphorins: From Angiogenesis to Inflammation in Rheumatoid Arthritis

    Jerome Avouac1, Sonia Pezet 2, Eloïse Vandebeuque 1 and Yannick Allanore 1, 1Paris Descartes University, Cochin Hospital, Rheumatology department, Paris, France, 2INSERM U1016, Paris, France

    Background/Purpose: Synovial neoangiogenesis is an early and crucial event to promote the development of the hyperplasic proliferative pathologic synovium in rheumatoid arthritis (RA). A recent…
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