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  • Abstract Number: 757 • 2017 ACR/ARHP Annual Meeting

    Plasmacytoid Dendritic Cells Activated through TLR8 Promote Systemic Sclerosis

    Marie-Dominique Ah Kioon1, Claudio Tripodo2, Alexandra Morquette3, Robert F. Spiera3, Jessica K. Gordon3 and Franck J. Barrat1, 1Autoimmunity and Inflammation Program, Hospital for Special Surgery, New York, NY, 2Department of Health Science, Human Pathology, tumor immunology unit, Palermo, Italy, 3Rheumatology, Hospital for Special Surgery, New York, NY

    Background/Purpose: Plasmacytoid Dendritic Cells (PDCs) are the key cell type mediating TLR-induced inflammation in several autoimmune diseases such as lupus, dermatomyositis, lichen sclerosus and cutaneous…
  • Abstract Number: 758 • 2017 ACR/ARHP Annual Meeting

    Identification of Biomarkers Predictive of Pulmonary Arterial Hypertension in Systemic Sclerosis

    Kathleen D. Kolstad1, Tyson Holmes2, Yael Rosenberg-Hasson2, Andrew Sweatt3, Roham T. Zamanian4, Shufeng Li5, Virginia D. Steen6, Paul J. Utz7 and Lorinda Chung8, 1Rheumatology, Stanford University Medical Center, Stanford, CA, 2Stanford University Medical Center, Stanford, CA, 3Medicine, Division of Pulmonary and Critical Care, Stanford University Medical Center, Stanford, CA, 4Stanford University Medical Center, Palo Alto, CA, 5Dermatology, Stanford University School of Medicine, Stanford, CA, 6Rheumatology, MedStar Georgetown University Hospital, Washington, DC, 7Medicine, Stanford University School of Medicine, Stanford, CA, 8Rheumatology, Stanford University Medical Center, Palo Alto, CA

    Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, immune system dysregulation, and fibrosis. Pulmonary arterial hypertension (PAH) affects approximately 10% of…
  • Abstract Number: 759 • 2017 ACR/ARHP Annual Meeting

    Multi Dimensional Analysis of the Immunome in Systemic Sclerosis Reveals Functionally Related Abnormalities in MAIT and B Cell Compartments

    Bhairav Paleja1, Pavanish Kumar1, Suzan Saidin1, Ahmad Lajam2, Camillus Chua1, Liyun Lai1, Andrea Hsiu Ling Low2 and Salvatore Albani1, 1SingHealth Translational Immunology and Inflammation Centre (STIIC), Duke-NUS Medical School, Singapore, Singapore, 2Singapore General Hospital, Singapore, Singapore

    Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterised by excessive fibrosis of skin and internal organs, and vascular dysfunction. Association of T and B…
  • Abstract Number: 760 • 2017 ACR/ARHP Annual Meeting

    Antisense Long Noncoding RNA HAND2-AS1 and OTUD6B-AS1 Have Important Roles in the Pathogenesis of Systemic Sclerosis

    Miki Takata1, Elena Pachera1, Anastasiia Kozlova1, Astrid Jüngel1, Tobias Messemaker2, Jeska de Vries-Bouwstra2, Tom W.J. Huizinga3, Fina Kurreeman2 and Oliver Distler4, 1Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, 2Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, Leiden, Netherlands, 3Department of Rheumatology, LUMC, Leiden, Netherlands, Leiden, Netherlands, 4Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: Long noncoding RNAs (lncRNAs) represent a class of transcripts longer than 200 nucleotides that are not translated into proteins. In recent years, antisense (AS)…
  • Abstract Number: 761 • 2017 ACR/ARHP Annual Meeting

    Increased Expression of the TNF Superfamily Member LIGHT/TNFSF14 and Its Receptor (TNFRSF14) in Patients with Systemic Sclerosis

    Otylia Kowal-Bielecka1, Ewa Gindzienska-Sieskiewicz1, Oliver Distler2, Joanna Reszec3, Suzana Jordan2, Pawel Bielecki4, Andzrzej Sieskiewicz4, Agnieszka Sulik1 and Krzysztof Kowal5,6, 1Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, 2Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 3Department of Medical Pathomorphology, Department of Medical Pathomorphology, Medical University of Bialystok, Bialystok, Poland, 4Department of Otolaryngology, Medical University of Bialystok, Bialystok, Poland, 5Department of Allergology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, 6Department of Experimental Allergology and Immunology, Medical University of Bialystok, Bialystok, Poland

    Background/Purpose: The TNF Superfamily member LIGHT (TNFSF14) regulates immune response and angiogenesis. Moreover, recent studies indicate that interactions of LIGHT with its receptor, TNFRSF14, might…
  • Abstract Number: 762 • 2017 ACR/ARHP Annual Meeting

    M10, a Caspase Cleavage Product of the Hepatocyte Growth Factor Receptor, Downregulates Bone Morphogenetic Protein-9-Induced Smad1/5/8 Phosphorylation and Collagen Production in Human Lung Fibroblasts

    Atsushi Noguchi, Ilia Atanelishvili, Tanjina Akter, Richard M. Silver and Galina S. Bogatkevich, Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC

    Background/Purpose: We recently identified M10, a caspase cleavage product of the hepatocyte growth factor receptor, as an anti-fibrotic peptide that interacts with Smad2 and inhibits…
  • Abstract Number: 763 • 2017 ACR/ARHP Annual Meeting

    Mechanisms of Insulin-like Growth Factor-II-Mediated Fibrosis

    Sara Garrett1, Justin Thomas2, Eileen Hsu3 and Carol A. Feghali-Bostwick4, 1Medicine/Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, 2Eisenhower Medical Center, Rancho Mirage, CA, 3Kaiser Permanente Medical Group, Mclean, VT, 4Division of Rheumatology and Immunology, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, United States, Charleston, SC

    Background/Purpose: Previous work has shown that insulin-like growth factor (IGF)-II is increased in fibrosing lung diseases, including idiopathic pulmonary fibrosis (IPF) and scleroderma/systemic sclerosis (SSc)-associated…
  • Abstract Number: 764 • 2017 ACR/ARHP Annual Meeting

    Application of a Novel Computational Approach to Identify New Targets and Pathways for Therapeutic Intervention in Scleroderma

    Elma Kurtagic, Joel Pradines, Anthony Manning and Ishan Capila, Research, Momenta Pharmaceuticals, Inc., Cambridge, MA

    Background/Purpose: Systemic Sclerosis (SSc) is a complex autoimmune disease with chronic progressive course and high interpatient variability. It is characterized by inflammation, vascular dysfunction and…
  • Abstract Number: 765 • 2017 ACR/ARHP Annual Meeting

    Proteomic Analysis of Scleroderma Associated Joint Disease

    Chan Kim1, Julio Mantero2, Robert A. Lafyatis3 and Robert W. Simms4, 1Rheumatology and Clinical Epidemiology, Boston University School of Medicine, Boston, MA, 2Arthritis Center, Boston University School of Medicine, Boston, MA, 3Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, 4Rheumatology, Boston University School of Medicine, Boston, MA

    Background/Purpose: The pathophysiology of joint disease in scleroderma, a heterogeneous multisystem disease mostly characterized by fibrosis, is unknown.  We performed proteomic analysis of serum in…
  • Abstract Number: 766 • 2017 ACR/ARHP Annual Meeting

    Increased Plasma Levels of Hsp90 Are Associated with More Severe Organ Involvement in Patients with Systemic Sclerosis

    Hana Storkanova1, Sabina Oreska1, Maja Spiritovic1,2, Karel Pavelka1, Jiri Vencovsky1, Jörg Distler3, Ladislav Senolt1, Radim Becvar1 and Michal Tomcik1, 1Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, 2Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, Prague, Czech Republic, 3Department of Internal Medicine III and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, Erlangen, Germany

    Background/Purpose: Our previous study demonstrated that Heat shock protein 90 (Hsp90) is overexpressed in the skin of patients with systemic sclerosis (SSc), in cultured SSc…
  • Abstract Number: 767 • 2017 ACR/ARHP Annual Meeting

    Elevated MeCP2 Expression in Diffuse Cutaneous Systemic Sclerosis Dermal Fibroblasts Is Associated with Anti-Fibrotic Effects

    Ye He1, Pei-Suen Tsou2, Dinesh Khanna3 and Amr H Sawalha2, 1Rheumatology, University of Michigan, Ann Arbor, MI, 2Division of Rheumatology, University of Michigan, Ann Arbor, MI, 3University of Michigan, Ann Arbor, MI

    Background/Purpose: Systemic Sclerosis (SSc) is a multisystem autoimmune connective tissue disorder characterized by vascular injury and fibrosis of the skin and internal organs. Methyl-CpG-binding protein…
  • Abstract Number: 768 • 2017 ACR/ARHP Annual Meeting

    A Positive Feedback Loop between Estrogen and IL-6 Leads to Fibrosis in Human Skin

    DeAnna Baker Frost1 and Carol A. Feghali-Bostwick2, 1Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, 2Division of Rheumatology and Immunology, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, United States, Charleston, SC

    Background/Purpose: Systemic Sclerosis (SSc) is a disease characterized by an increase in the synthesis of extracellular matrix (ECM) components in various organs, including the skin,…
  • Abstract Number: 769 • 2017 ACR/ARHP Annual Meeting

    Proteomic Analysis of Human Fibroblasts in Systemic Sclerosis Reinforces the Role of Transforming Growth Factor-ß and Points Toward Epidermal Growth Factor Receptor / Phosphatidylinositol 3 Kinase Pathway Inhibition

    Benjamin Chaigne1, Guilhem Clary2, Morgane Le Gall3, Nicolas Dumoitier4, Sebastien Lofek5, Philippe Chafey2, Pia Moinzadeh6, Thomas Krieg7, Christopher Denton8 and Luc Mouthon9, 1Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes Rares d’Ile de France, hôpital Cochin, DHU Authors, Assistance Publique-Hôpitaux de Paris, Paris, France, 2INSERM U1016, Institut Cochin,, Paris, France, 3INSERM U1016 Institut Cochin, Paris, France, 4INSERM U1016, Institut Cochin, Equipe Neutrophiles et Vascularites, Paris, France, 5INSERM U1016, paris, France, 6Department of Rheumatology, UCL Division of Medicine, London, United Kingdom, 7Universität zu Köln, Köln, Germany, 8Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom, 9Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France ;Université Paris Descartes Sorbonne Paris, Paris, France

    Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disorder characterized by autoimmunity, vasculopathy and fibrosis. Fibrosis is due to an exaggerated activation of…
  • Abstract Number: 770 • 2017 ACR/ARHP Annual Meeting

    Integrating Analysis of Skin RNA in Situ Hybridization Using Rnascope and Whole Skin Gene Expression in Systemic Sclerosis Skin to Localize Key Pathogenic Drivers of Skin Fibrosis

    Corrado Campochiaro1, Emma C. Derrett-Smith1, Voon H. Ong2, Gail Pearse3, Katherine Nevin3, Shaun Flint3, Mary Morse3, Nicolas Wisniacki4 and Christopher Denton5, 1Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, London, United Kingdom, 2Rheumatology, UCL Division of Medicine, London, United Kingdom, 3GlaxoSmithKline, Stevenage, United Kingdom, 4ImmunoImflammation, GlaxoSmithKline, Stevenage, United Kingdom, 5Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom

    Background/Purpose: Skin gene expression profiling can distinguish SSc from normal skin and can detect different subsets of disease. Previous studies have reported a cross-sectional relationship…
  • Abstract Number: 771 • 2017 ACR/ARHP Annual Meeting

    Serological Biomarkers of Collagen Formation Is Increased in Systemic Sclerosis

    Pernille Juhl1, Mette Mogensen2, Line Iversen2, Tonny Karlsmark2, Morten Karsdal3, Anne-C. Bay-Jensen4 and Anne Sofie Siebuhr5, 1Nordic Bioscience, Herlev, Denmark, 2Department of Dermatology, Bispebjerg Hospital, Copenhagen, Denmark, 3Biomarkers and Reseacrh, Nordic Bioscience, Herlev, Denmark, 4Biomarkers and Reseach, Nordic Bioscience, Herlev, Denmark, 5Biomarkers and Research, Nordic Bioscience, Herlev, Denmark, Herlev, Denmark

    Background/Purpose: Systemic sclerosis (SSc) is a multisystem, autoimmune disease characterized by immune dysregulation, vasculopathy and excessive fibrosis of the skin and internal organs. Fibrosis is…
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