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  • Abstract Number: 1859 • 2016 ACR/ARHP Annual Meeting

    Fucosyltransferase-1 Mediated Fucosylation of TGF-βR1 Is Critical to TGF-β Signaling in Scleroderma and in Bleomycin-Induced Fibrosis

    W. Alexander Stinson1, Pei-Suen Tsou1,2, Yuxuan Du3, Huadong Cui1, Ellen Cealey3, Nicholas Lepore4, Ray A. Ohara1, Gautam Edhayan1, Sarah Arwani1, Rachel Morgan1, Dinesh Khanna1,2, David A. Fox1 and M. Asif Amin5, 1Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI, 2University of Michigan Scleroderma Program, Ann Arbor, MI, 3Rheumatology, Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI, 4University of Michigan, Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI, 5Internal Medicine, Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI

    Background/Purpose:  Systemic sclerosis (SSc) is a connective tissue disease characterized by systemic fibrosis. The dysregulation of transforming growth factor-β (TGF-β) signaling causes proliferation of myofibroblasts…
  • Abstract Number: 1860 • 2016 ACR/ARHP Annual Meeting

    Activating Transcription Factor 3 – a New Linkage Between Vasculopathy and Organ Fibrosis in Systemic Sclerosis

    Thomas Wohlfahrt1, Alina Soare2, Tatjana Mallano2, Morgane Gourlaouen3, Stephen Moss3, Britta Maurer4, Oliver Distler4, Tsonwin Hai5, Georg Schett2, Jörg Distler2 and Andreas Ramming2, 1Department of Internal Medicine 3, Rheumatology and Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 2Department of Internal Medicine 3 and Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany, 3Institute of Ophthalmology, Department of Cell Biology, University College London, London, United Kingdom, 4Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 5Department of Molecular and Cellular Biochemistry, The Ohio State University, Colombus, OH

    Background/Purpose: Since vascular manifestations such as Raynaud’s phenomenon and morphological changes on nailfold capillaroscopy often precede the onset of other clinical manifestations of systemic sclerosis…
  • Abstract Number: 1861 • 2016 ACR/ARHP Annual Meeting

    Modelling the Interaction Between Disease Microenvironment and Mesenchymal Cells in Systemic Sclerosis

    Zeinab Taki1, Bahja Ahmed Abdi2, Henrique Rosario3, Sara Zafar4, Amy Hart3, Shiwen Xu5, Christopher Denton6, David Abraham6 and Richard J. Stratton7, 1Department of Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 2Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 3Centre of Rheumatology and Connective Tissue disease, University College London, London, United Kingdom, 4Centre of Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 5Division of Medicine, ​Centre for Rheumatology and Connective tissue disease, University College London, London, United Kingdom, 6Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 7Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom

    Background/Purpose: Systemic Sclerosis (SSc) has complex aetiology with many potential driving forces, one of which is the microenvironment in lesional skin, in which resident mesenchymal stem…
  • Abstract Number: 1862 • 2016 ACR/ARHP Annual Meeting

    Modelling Healthy and Scleroderma Fibrotic Skin in Vitro: Mechanical Stress Alters Macrophage Cytokine Expression and Triggers Signalling Via the Mechano-Sensing Transcription Factor Myocardin-Related Transcription Factor-a

    Angela Tam1, Shiwen Xu1, Henry Lopez1, Korsa Khan2, Bahja Ahmed Abdi3, Henrique Rosario4, Nikita Arumalla2, Mark Gibson2, Christopher Denton2, David Abraham2, Barbara D Smith5 and Richard J Stratton2, 1Division of Medicine, ​Centre for Rheumatology and Connective tissue disease, University College London, London, United Kingdom, 2Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 3Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 4Division of Medicine, Centre of Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 5Boston University School of Medicine, Boston, MA

    Background/Purpose: Skin involvement is one of the most prominent clinical features in scleroderma. There is a marked contrast in mechanical stiffness between healthy forearm skin…
  • Abstract Number: 1863 • 2016 ACR/ARHP Annual Meeting

    Non-Invasive Investigation of Perfusion, Microvascular Structure, Erythema, Oxidative Stress and Oxygenation in Healthy Controls and in Patients with Primary and Secondary Raynaud’s Phenomenon

    Andrea Murray1,2, Tonia Moore3, Joanne Manning4, Ian Poxon2, Graham Dinsdale1, Michael Berks5, Sarah Leggett6, Mark Dickinson2 and Ariane L. Herrick1, 1Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Manchester, United Kingdom, 2Photon Science institute, University of Manchester, Manchester, United Kingdom, 3Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Salford, United Kingdom, 4Rheumatology Department, Salford Royal NHS Foundation Trust, Salford, United Kingdom, 5Centre for Imaging Sciences, University of Manchester, Institute of Population Health, Manchester, United Kingdom, 6Arthritis Research UK Centre for Epidemiology, The University of Manchester, Manchester, United Kingdom

    Background/Purpose: It is well established that measurements of cutaneous microvascular dysfunction (measured with laser Doppler imaging [LDI]) and structural abnormality (measured with nailfold capillaroscopy [NC])…
  • Abstract Number: 1864 • 2016 ACR/ARHP Annual Meeting

    No Evidence of Association of ATP8B4 F436L missense Variant in a Large Systemic Sclerosis Cohort

    Elena Lopez-Isac1, Lara Bossini-Castillo2, Ana B Palma2, Shervin Assassi3, Carmen Pilar Simeón4, Norberto Ortego Centeno5, Esther Vicente-Rabaneda6, Carlos Tolosa7, Manuel Rubio-Rivas8, Jose Andres Roman Ivorra9, Lorenzo Beretta10, Gianluca Moroncini11, Nicolas Hunzelmann12, Joerg HW Distler13, Gabriela Riekemasten14, Jeska K. de Vries-Bouwstra15, Alexandre E. Voskuyl16, Timothy R.D.J. Radstake17, Ariane L. Herrick18, Christopher Denton19, Carmen Fonseca20, Maureen D Mayes3 and Javier Martín1, 1Institute of Parasitology and Biomedicine López-Neyra, IPBLN-CSIC, Granada, Spain, 2Cellular Biology and Immunology, Institute of Parasitology and Biomedicine López-Neyra (CSIC), Granada, Spain, 3Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, 4Internal Medicine, Hospital Universitari Vall d'Hebron, Barcelona, Spain, 5Internal Medicine, Hospital Clínico San Cecilio, Granada, Spain, 6Rheumatology, H.U. La Princesa, Madrid, Spain, 7Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Barcelona, Spain, 8Department of Internal Medicine, Hospital Universitario de Bellvitge, Barcelona, Spain, 9Department of Rheumatology, Hospital Universitario y Politecnico La Fe, Valencia, Spain, 10Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 11Dipartimento di Scienze mediche e Chirurgiche, Università politecnica delle Marche and Ospedali Riuniti, Ancona, Italy, 12Department of Dermatology, University of Cologne, Cologne, Germany, 13Internal Medicine 3, University of Erlangen, Erlangen, Germany, 14Department of Rheumatology, University of Lübeck, Luebeck, Germany, 15Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 16Department of Rheumatology, VU University medical center, Amsterdam, Netherlands, 17Laboratory of Translational Immunology, UMC Utrecht, Utrecht, Netherlands, 18Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Manchester, United Kingdom, 19Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 20Centre for Rheumatology, Royal Free and University College Medical School, London, United Kingdom

    Background/Purpose: Systemic sclerosis (SSc) is a complex autoimmune disease with heterogeneous clinical manifestations. Over the past seven years our knowledge of the SSc genetic component…
  • Abstract Number: 1865 • 2016 ACR/ARHP Annual Meeting

    Urinary Cell Adhesion Molecules As Markers of Renal Involvement in Systemic Sclerosis

    Edward Stern1, Voon H. Ong2, Aine Burns3, Robert Unwin4 and Christopher Denton5, 1UCL Division of Medicine, London, United Kingdom, 2Rheumatology, UCL Division of Medicine, London, United Kingdom, 3Nephrology, Royal Free Hospital, London, United Kingdom, 4Nephrology, UCL Division of Medicine, London, United Kingdom, 5Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom

    Background/Purpose: Renal involvement in systemic sclerosis (SSc) includes scleroderma renal crisis as well as progressive organ fibrosis. Detection and management of these disease complications is…
  • Abstract Number: 1866 • 2016 ACR/ARHP Annual Meeting

    Human and Experimental Tracheal Stenosis Is Characterized By a TGF-β-Dependent Fibrotic Component

    Alicia Usategui1, Juan L. Antón-Pacheco2, Carmen M. García-Herrero1, Manuel J. Del Rey1, Vanessa Miranda1, Iván Martínez3, Antonio P Gámez3 and Jose L. Pablos1, 1Grupo de Enfermedades Inflamatorias y Autoinmunes, Instituto de Investigación Hospital 12 de Octubre (i+12), Madrid, Spain, 2Servicio de Cirugía Pediátrica, Hospital 12 de Octubre, Madrid, Spain, 3Servicio de Cirugía Torácica, Hospital 12 de Octubre, Madrid, Spain

    Background/Purpose: Acquired tracheal stenosis (ATS) is an unusual disease secondary to inflammatory diseases or to prolonged mechanical trauma. Tracheal mucosa undergoes inflammation and granulation tissue…
  • Abstract Number: 1867 • 2016 ACR/ARHP Annual Meeting

    An Abnormal Nailfold Capillaroscopy Pattern Is Common in Patients with Connective Tissue Disease and Is Associated with Pulmonary and Oesophageal Involvement, Even in the Absence of Systemic Sclerosis

    Anniek M van Roon1, Cato C Huisman1, Arie M van Roon1, Alja J Stel2, Andries J Smit1, Hendrika Bootsma2 and Douwe J Mulder1, 1Internal Medicine - division Vascular Diseases, University of Groningen, University Medical Center Groningen, Groningen, Netherlands, 2Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, Netherlands

    Background/Purpose: RP is a common symptom of CTD. Nailfold capillary microscopy (NCM) has previously been shown to be associated with disease severity and internal organ…
  • Abstract Number: 1868 • 2016 ACR/ARHP Annual Meeting

    Rates and Predictors of Physical Therapy and Occupational Therapy Utilization in Systemic Sclerosis: A Scleroderma Patient-Centered Intervention Network Cohort Study

    Karima Becetti1, Jessica K. Gordon1, Joseph Nguyen2, Carol Mancuso3, Linda Kwakkenbos4,5, Marie-Eve Carrier5, Brett D. Thombs4,5, Robert F. Spiera1 and SPIN Investigators, 1Rheumatology, Hospital for Special Surgery, New York, NY, 2Epidemiology and Biostatistics, Hospital for Special Surgery, New York, NY, 3Medicine, Hospital for Special Surgery, New York, NY, 4McGill University, Montreal, QC, Canada, 5Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada

    Background/Purpose:  Systemic sclerosis (SSc) is characterized by significant disability due to musculoskeletal involvement. Physical and occupational therapy (PT/OT) have been suggested to improve hand function,…
  • Abstract Number: 1869 • 2016 ACR/ARHP Annual Meeting

    Mental Health Care in Systemic Sclerosis; Rates of Utilization and Associated Factors in the Scleroderma Patient-Centered Intervention Network Cohort

    Karima Becetti1, Jessica K. Gordon1, Joseph Nguyen2, Carol Mancuso3, Linda Kwakkenbos4,5, Marie-Eve Carrier4,5, Brett D. Thombs4,5, Robert F. Spiera1 and SPIN Investigators, 1Rheumatology, Hospital for Special Surgery, New York, NY, 2Epidemiology and Biostatistics, Hospital for Special Surgery, New York, NY, 3Medicine, Hospital for Special Surgery, New York, NY, 4McGill University, Montreal, QC, Canada, 5Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada

    Background/Purpose:  Systemic sclerosis (SSc) is characterized by high disfigurement, morbidity, and mortality. It carries significant psychosocial impact including depression, anxiety and body image distress. However,…
  • Abstract Number: 1870 • 2016 ACR/ARHP Annual Meeting

    First Pilot Study of an Implantable Loop Recorder (ILR) in Systemic Sclerosis Detects Significant Cardiac Arrhythmias with CMR Abnormalities

    Lesley-Anne Bissell1, Bianca Dumitru1, Giuseppina Abignano1, Bara Erhayiem2, Graham Fent2, Peter Swoboda2, Adam McDiarmid2, John Greenwood2, Francesco Del Galdo1, Jacqueline Andrews1, Sven Plein2, Lee Graham2 and Maya Buch1, 1Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds and NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 2Multidisciplinary Cardiovascular Research Centre & The Division of Cardiovascular and Diabetes Research, LIGHT, University of Leeds, Leeds, United Kingdom

    Background/Purpose: SSc-cardiomyopathy associated conduction abnormalities carry a poor prognosis, but their pathogenesis is unclear. Early detection to prevent complications is essential. ILRs are well-established in…
  • Abstract Number: 1871 • 2016 ACR/ARHP Annual Meeting

    Predicting Vascular Complications in Systemic Sclerosis: A Prospective Cohort Study

    Christopher A. Mecoli1, Ami A. Shah2, Francesco Boin3, Fredrick M. Wigley4 and Laura K. Hummers1, 1Johns Hopkins University School of Medicine, Baltimore, MD, 2Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 3Rheumatology, University California San Francisco, San Francisco, CA, 4Rheum Div/Mason F Lord, Johns Hopkins University School of Medicine, Baltimore, MD

    Background/Purpose: Vascular disease is of fundamental importance in the pathogenesis of scleroderma. Two major vascular complications in scleroderma patients that cause substantial morbidity and mortality…
  • Abstract Number: 1872 • 2016 ACR/ARHP Annual Meeting

    The Effect of Cyclophosphamide on Pulmonary Function and Dependence on Disease Activity of Interstitial Lung Disease Associated with Systemic Sclerosis

    WMT van den Hombergh1, E. Teesselink1, HKA Knaapen-Hans1, FHJ van den Hoogen2, S.O. Simons3, J. Fransen4 and MC Vonk1, 1Rheumatology, Radboud University Medical Centre, Nijmegen, Netherlands, 2Rheumatology, Radboudumc, Nijmegen, Netherlands, 3Respiratory Medicine, Radboud University Medical Centre, Nijmegen, Netherlands, 4Department of Rheumatolgy, Radboudumc Nijmegen, Nijmegen, Netherlands

    THE EFFECT OF CYCLOPHOSPHAMIDE ON PULMONARY FUNCTION AND DEPENDENCE ON DISEASE ACTIVITY OF INTERSTITIAL LUNG DISEASE ASSOCIATED WITH SYSTEMIC SCLEROSIS W. M. T. Van Den…
  • Abstract Number: 1873 • 2016 ACR/ARHP Annual Meeting

    Longitudinal Analysis of Modified Rodnan Skin Score in Systemic Sclerosis Using Group-Based Trajectory Modelling

    Emmanuel Ledoult1,2, Vincent Sobanski1,3,4,5, Luc Mouthon6,7,8, Hélène Béhal9, Christian Agard10, Jean-Christophe Lega11, Patrick Jego12, Yves-Michel Frances13, Gilles Kaplanski14,15, Jean-Robert Harle16, Sabine Berthier17, Boris Bienvenu18, Olivier Fain19, Arsene Mekinian20, Elisabeth Diot21, Robin Dhote22, Alain Le Quellec23, Zahir Amoura24,25, Noemie Le Gouellec26, Jean-Emmanuel Kahn27, Nadine Magy28, Marie-Hélène Balquet29, Grégory Pugnet30, Thomas Papo31, Pierre Kieffer32, Viviane Queyrel33, Jean-Baptiste Gaultier34, Denis Wahl35, Francois Maurier36, Emmanuel Chatelus37, Jean-Louis Pennaforte38, Olivier Aumaître39, Olivier Lidove40, Cristina Belizna41, Carine Boulon42, Marie-Elise Truchetet43, Jacques Pouchot44, Eric Auxenfants45, Anne-Laure Fauchais46, Bernard Imbert47, Eric Hachulla1,48,49,50, David Launay3,4,5,51 and the French Autoimmune and Autoinflammatory Rare Diseases Network (FAI2R), 1CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, 2CHU Lille, Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), F-59000 Lille, France, 3CHU Lille, Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), F-59000 Lille, France, Lille, France, 4Inserm, U995, F-59000 Lille, France, Lille, France, 5Univ. Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France, Lille, France, 6Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, Paris, France, 7National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 8INSERM U1016, Institut Cochin, Equipe Neutrophiles et Vascularites, Paris, France, 9Maison Régionale de la Recherche Clinique. CHU de Lille. F- 59000 Lille, France, Lille, France, 10Internal Medicine Department, Nantes University Hospital, Nantes, France, 11Internal Medicine, Lyon Sud Hospital, Hospices Civils de Lyon, University of Lyon, Lyon, France, 12CHU de Rennes - Internal Medicine, Rennes, France, 13Médecine gériatrique - Hôpital Nord - F-13000 Marseilles, Marseilles, France, 14Aix-Marseille Université - Internal Medicine hopital conception - F-13000 Marseilles, Marseille, France, 15INSERM U608, Marseille, France, 16Service de médecine interne et immunologie clinique / Hôpital de la Timone, Marseilles, France, 17Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, 18Caen University Hospital, Caen, France, 19Service de médecine interne. Hôpital Saint-Antoine., Paris, France, 20Internal Medicine, DHUi2B Saint Antoine Hospital, paris, France, 21Pôle médecine interne et gériatrique, pneumologie CHRU de Tours - Hôpital Bretonneau, Tours, France, 22Service de médecine interne. Hôpital Avicenne, Paris, France, 23Division of internal Medicine, Hôpital Saint-Eloi, Centre Hospitalier Universitaire de Montpellier, Montpellier, Montpellier, France, 24Internal Medecine - Centre de Référence National pour les Lupus et et le Syndrome des Antiphospholipides, Internal Medecine - Centre de Référence National pour les Lupus et et le Syndrome des Antiphospholipides, Pitié-Salpêtrière Hospital (AP-HP), Paris, France, 25Department of Internal Medicine, Pitié-Salpêtrière Hospital, Paris, France, 26Internal Medicine, Lille, France, 27Internal Medicine, Foch Hospital, Suresnes, France, 28Médecine Interne. CHU Besancon, Besancon, France, 29Médecine Interne - CH Lens, Lens, France, 30Department of Internal Medicine, Toulouse University Hospital, University of Toulouse, INSERM UMR 1027, Toulouse, France, 31Bichat University Hospital - Internal Medicine, Paris, France, 32Hopital Emile Muller - Medecine Interne, Mulhouse Cedex 1, France, 33Service de médecine interne - CHU de Nice. Hopital de l'Archet., Nice, France, 34Pole de Gériatrie et Médecine interne - CHU Saint-Etienne, Saint-Etienne, France, 35CHU de Nancy - Service de médecine interne - Unité de Médecine vasculaire Institut lorrain du coeur et des vaisseaux Louis Mathieu, Nancy, France, 36Centre de Compétences des Maladies Systémiques Rares - CHU Metz, metz, France, 37Department of Rheumatology, Strasbourg University Hospital, Strasbourg, France, 38Service de médecine interne Pôle Médecines CHU de Reims - Hôpital Robert Debré, Reims, France, 39CHU Pitié-Salpêtrière - Department of Internal Medicine 2. Referal center for SLE/APS, Paris, France, 40Service de Médecine Interne-Rhumatologie Hôpital de la Croix St Simo, Paris, France, 41Angers University hospital - Internal Medicine, Angers, France, 42Service de médecine interne et vasculaire - CHU Bordeaux, Bordeaux, France, 43CHU de Bordeaux - Service de médecine interne, Bordeaux, France, 44Internal Medicine Department, European Hospital Georges Pompidou, Paris, France, 45CH Roubaix - Médecine interne, Roubaix, France, 46Department of Internal Medicine, CHU de Limoges, Limoges, France, 47CHU, Grenoble, Grenoble, France, 48CHU Lille, Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), F-59000, France, 49Univ. Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France, 50Inserm, U995, F-59000 Lille, France, 51CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, Lille, France

    Background/Purpose: The modified Rodnan skin score (mRSS) is a clinical evaluation score of the extent of cutaneous fibrosis in systemic sclerosis (SSc). mRSS has been…
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All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

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