ACR Meeting Abstracts

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  • Abstract Number: 1644 • 2019 ACR/ARP Annual Meeting

    Effects of Nintedanib in Patients with Diffuse and Limited Cutaneous Systemic Sclerosis and Interstitial Lung Disease: Subgroup Analysis of the SENSCIS Trial

    Masataka Kuwana1, Kristin Highland 2, Martina Gahlemann 3, Christopher Denton 4, Aryeh Fischer 5, Maureen Mayes 6, Virginia Steen 7, Dinesh Khanna 8, Yannick Allanore 9, Mannaig Girard 10, Margarida Alves 11, Susanne Stowasser 11 and Oliver Distler 12, 1Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, 2Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, 3Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, 4University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, 5University of Colorado School of Medicine, Denver, Colorado, USA, Denver, CO, 6Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, 7Georgetown University, Washington, D.C., USA, Georgetown, 8Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, 9Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, 10Boehringer Ingelheim France S.A.S., Reims, France, Reims, France, 11Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, 12Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland

    Background/Purpose: Patients with diffuse cutaneous systemic sclerosis (dcSSc) are at greater risk of developing interstitial lung disease (ILD) than patients with limited cutaneous systemic sclerosis…
  • Abstract Number: 1645 • 2019 ACR/ARP Annual Meeting

    Comparison of Automated Capture and Analysis System of Sublingual Microvessels and Nailfold Videocapillarscopy for Microvascular Assessment in Systemic Sclerosis

    Mislav Radic1, Julie Thomas 2 and Tracy Frech 3, 1University Hospital Split, Split, Croatia, 2University of Utah, Division of Rheumatology, Salt Lake City, UT, 3Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT

    Background/Purpose: Similar to nailfold videocapillaroscopy (NVC), automated capture and analysis of sublingual microvessel segments (intravital microscopy) can define vasculopathy, and distinguish systemic sclerosis (SSc) patients…
  • Abstract Number: 1646 • 2019 ACR/ARP Annual Meeting

    Incidence Rate and Prevalence of Valvular Heart Disease in Systemic Sclerosis: A Retrospective Cohort Study from a Single Institution

    Mamoun Elnagar1, Yasser Radwan 1, Mohamed Elwazir 2, Reto Kurmann 2, Rekha Mankad 1, Thomas Osborn 1, Ashima Makol 1, Leroy Griffing 3, Cynthia Crowson 2 and Alicia Hinze 1, 1Mayo Clinic Minnesota, Rochester, MN, 2Mayo Clinic Minnesota, Rochester, 3Mayo Clinic Arizona, Scottsdale

    Background/Purpose: Systemic sclerosis (SSc) associated heart involvement remains ill-defined, with non-ischemic systolic heart failure, conduction defects, and arrhythmias often included in the definition in clinical…
  • Abstract Number: 1647 • 2019 ACR/ARP Annual Meeting

    The Collaborative National Quality and Efficacy Registry for Scleroderma: Data Completion Outcomes from a Multicenter United States Cohort Using Guideline-Based Registry Practices

    Victoria Shanmugam1, Tracy Frech 2, Virginia Steen 3, Laura Hummers 4, Ami Shah 5, Elana Bernstein 6, Dinesh Khanna 7, Jessica Gordon 8, Flavia Castelino 9, Lorinda Chung 10, Faye Hant 11, Emily Startup 12, John VanBuren 12, Luke Evnin 13 and Shervin Assassi 14, 1George Washington University, Georgetown, DC, 2Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, 3Georgetown University, Washington, D.C., USA, Georgetown, 4Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, 5Johns Hopkins Hospital, Baltimore, MD, 6Division of Rheumatology, Columbia University, New York, NY, 7Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, 8Hospital for Special Surgery, New York, NY, 9Harvard University, Boston, MA, 10Stanford University, Palo Alto, CA, 11Medical University of South Carolina, Charleston, SC, 12University of Utah, Salt Lake City, UT, 13Scleroderma Research Foundation, San Francisco, CA, 14Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX

    Background/Purpose: The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5…
  • Abstract Number: 1648 • 2019 ACR/ARP Annual Meeting

    Systemic Sclerosis Auto-antibody Profiles Predict Interstitial Lung Disease Onset but Not Progression

    Boyang Zheng1, Mianbo Wang 2, Marvin Fritzler 3, May Choi 3, Murray Baron 4 and Marie Hudson 5, 1Jewish General Hospital, McGill University, Montreal, QC, Canada, 2Lady Davis Institute, Jewish General Hospital, Montreal, QC, Canada, 3Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, 4Jewish General Hospital, McGill University, Montreal, Canada, 5Jewish General Hospital, Lady Davis Institute for Medical Research, and Department of Medicine, McGill University, Montreal, QC, Canada

    Background/Purpose: Interstitial lung disease (ILD) is frequent manifestation and a leading cause of mortality in systemic sclerosis (SSc). Some SSc-related autoantibodies (aAbs), especially anti-topoisomerase I…
  • Abstract Number: 1649 • 2019 ACR/ARP Annual Meeting

    Mycophenolate Mofetil and Cyclophosphamide Improve Health-Related Quality of Life in Patients with Systemic Sclerosis Who Participated in SLS II

    Elizabeth Volkmann1, Donald Tashkin 2, Michael Roth 3, Holly LeClair 2, Philip Clements 2, Daniel Furst 4, Robert Elashoff 2 and Dinesh Khanna 5, 1University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, 2University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, 3University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 4University of California, Los Angeles, CA, 5Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor

    Background/Purpose: Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). The majority of studies investigating novel therapies for…
  • Abstract Number: 1650 • 2019 ACR/ARP Annual Meeting

    Baseline Skin Score, Older Age and CD34-selection Influence the Outcome of Autologous Stem Cell Transplantation for Systemic Sclerosis – Results of a Prospective Non-interventional Study from the European Society for Blood & Marrow Transplantation (EBMT)

    Joerg Henes1, Maria Carolina Oliveira 2, Myriam Labopin 3, Manuela Badoglio 4, Hans Ulrich Scherer 5, Nicoletta Del Papa 6, Thomas Daikeler 7, Marc Schmalzing 8, Roland Schroers 9, Thierry Martin 10, Grégory Pugnet 11, Belinda Simoes 12, David Michonneau 13, Erik Marijt 14, Bruno Lioure 15, Jacques Olivier Bay 16, John Snowden 17, Montserrat Rovira 18, Anne Huynh 19, Francesco Onida 20, Lothar Kanz 1, Zora Marjanovic 21 and Dominique Farge 22, 1University Hospital Tuebingen, Department of Internal Medicine II (Oncology, Haematology, Immunology and Rheumatology), Tuebingen, Germany, 2Ribeirão Preto Medical School, University of São Paulo, Sao Paulo, Brazil, 3EBMT Paris Study Office, Saint Antoine Hospital, Université Pierre et Marie Curie, Paris, France, 4EBMT Paris Study Office, Hôpital St Antoine, Paris, France, 5Leiden University Medical Center, Department of Rheumatology, Leiden, Netherlands, 6Scleroderma Clinic, Osp. G. Pini, Department of Rheumatology, Milan, Italy, 7University and University Hospital of Basel, Department of Rheumatology, Basel, Switzerland, 8Universitätsklinikum Würzburg, Department of Rheumatology/ Clinical Immunology, Wuerzburg, Germany, 9University Hospital of Bochum, Med. Klinik, Bochum, Germany, 10Hôpitaux Universitaires de Strasbourg Service de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-Immunes Systémiques Rares Est-Sud-Ouest (RESO), Strasbourg, France, 11CHU de Toulouse, Hôpital Purpan, Service de Médecine Interne, Toulouse, France, 12Ribeirão Preto Medical School, University of São Paulo, Division of Hematology, Sao Paulo, Brazil, 13Hôpital Saint Louis & Université Paris 7, Denis Diderot, Department of Hematology, Paris, France, 14Leiden University Medical Center, Department of Hematology, Leiden, Netherlands, 15Strasbourg University Hospital, Department of Hematology, Strasbourg, France, 16CHU de Clermont Ferrand, Department of Hematology, Clermont Ferrand, France, 17Department of Haematology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom, 18Hospital Clínic of Barcelona, Department of Haematology, Barcelona, Spain, 19UCT Oncopole, Department of Haematology, Toulouse, France, 20Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, University of Milan, Milan, Italy, 21Saint Antoine Hospital, Department of Haematology, Paris, France, 22Hôpital Saint-Louis, AP-HP Assistance Publique des Hôpitaux de Paris, Centre de Référence des Maladies Auto-Immunes Systémiques Rares d’Ile-de-France, Filière ‘FAI2R’, Unité de Médecine Interne: Maladies Auto-immunes et Pathologie Vasculaire (UF 04), Université de Paris, EA 3518, Paris, France; Department of Medicine, McGill University, Montreal, QC, Canada, Paris, France

    Background/Purpose: TThree randomized controlled trials in systemic sclerosis (SSc) demonstrated that autologous hematopoietic stem cell transplantation (aHSCT) is superior to standard cyclophosphamide therapy. This EBMT…
  • Abstract Number: 1651 • 2019 ACR/ARP Annual Meeting

    Multicenter Double-Blind, Proof-of-Concept, Randomized Placebo-Controlled Trial of Riociguat in Systemic Sclerosis-associated Digital Ulcers

    Vivek Nagaraja1, Cathie Spino 1, Pei-Suen Tsou 1, Robyn Domsic 2, Robert Lafyatis 2, Tracy Frech 3, Jessica Gordon 4, Virginia Steen 5 and Dinesh Khanna 6, 1University of Michigan, Ann Arbor, MI, 2University of Pittsburgh, Pittsburgh, PA, 3Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, 4Hospital for Special Surgery, New York, NY, 5Georgetown University, Washington, D.C., USA, Georgetown, 6Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor

    Background/Purpose: The soluble guanylate cyclase stimulator riociguat (RIO) is a vasodilator with efficacy in patients with pulmonary arterial hypertension associated with connective tissue disease. Our…
  • Abstract Number: 1652 • 2019 ACR/ARP Annual Meeting

    Anticentromere Antibody Levels and Isotypes Associate with Disease Severity in Systemic Sclerosis

    Nina van Leeuwen1, Jaap Bakker 1, Annette Grummels 1, Corrie Wortel 1, Suzana Jordan 2, Oliver Distler 3, Håvard Fretheim 4, Anna Maria Hoffmann-Vold 5, Hans Ulrich Scherer 6, René Toes 1, Thomas Huizinga 1 and Jeska de Vries-Bouwstra 1, 1Leiden University Medical Center, Leiden, Netherlands, 2Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, 3Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, 4University Hospital Oslo, Oslo, Norway, 5Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 6Leiden University Medical Center, Department of Rheumatology, Leiden, Netherlands

    Background/Purpose: Although some studies suggest a possible association between clinical characteristics and isotypes of anticentromere antibodies (ACA) in patients with systemic sclerosis (SSc), characteristics of…
  • Abstract Number: 1653 • 2019 ACR/ARP Annual Meeting

    Efficacy and Safety of Romilkimab in Diffuse Cutaneous Systemic Sclerosis (dcSSc): A Randomized, Double-Blind, Placebo-Controlled, 24-week, Proof of Concept Study

    Yannick Allanore1, Christopher Denton 2, Dinesh Khanna 3, Christina Soubrane 4, Corinne Esperet 4, Frederic Marrache 4, Raphael Bejuit 4, Amel Lahmar 5 and Peter Wung 5, 1Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, 2University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, 3Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, 4Sanofi, Chilly Mazarin, France, 5Sanofi, Bridgewater, NJ

    Background/Purpose: Systemic sclerosis (SSc) is a progressive, multi-organ disease with limited treatment options. Interleukin-4 (IL-4) and IL-13 have been implicated in the general fibrotic pathway…
  • Abstract Number: 1654 • 2019 ACR/ARP Annual Meeting

    Esophageal Dilation and Other Clinical Factors Associated with Pulmonary Function Decline in Patients with Systemic Sclerosis

    Kimberly Showalter1, Aileen Hoffmann 2, Carrie Richardson 3, Kimberly Koloms 2, Gerald Rouleau 2, David Aaby 2, Julia (Jungwha) Lee 4, Jane Dematte 2, Rishi Agrawal 2, Xiaoping Wu 5, Rowland Chang 4 and Monique Hinchcliff 6, 1Hospital for Special Surgery, New York, NY, 2Northwestern University Feinberg School of Medicine, Chicago, 3Rush University, Chicago, 4Northwestern University Feinberg School of Medicine, Chicago, IL, 5New York Presbyterian/Weill Cornell, New York, 6Yale University, Section of Rheumatology, Allergy and Immunology, New Haven, CT

    Background/Purpose: Prior work has shown that Scl-70 positive autoantibody status and baseline c-reactive protein (CRP) are associated with more rapid pulmonary function decline in patients…
  • Abstract Number: 1655 • 2019 ACR/ARP Annual Meeting

    Histologic Features Correlate with the Modified Rodnan Skin Score, Serum Inflammatory Markers, and Patient Reported Outcomes in Patients with Early, Diffuse Cutaneous Systemic Sclerosis

    Kimberly Showalter1, Cynthia Magro 2, Dana Orange 3, Yaxia Zhang 4, Phaedra Agius 5, Jackie Finik 6, Robert Spiera 1 and Jessica Gordon 1, 1Hospital for Special Surgery, New York, NY, 2Weill Cornell Medicine, New York, 3Rockefeller University, New York, 4Hospital for Special Surgery, New York, 5New York Genome Center, New York City, 6Hospital for Special Surgery, New York, NY, New York, NY

    Background/Purpose: The association between systemic sclerosis (SSc) skin histology and clinical findings is not fully characterized. In two SSc trials, we developed a scoring system…
  • Abstract Number: 1656 • 2019 ACR/ARP Annual Meeting

    Health-Related Quality of Life in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Impact of Lung Function on Patient-Reported Outcomes in a Randomized Phase III Trial

    Michael Kreuter 1, Anna Maria Hoffmann-Vold 2, Marco Matucci-Cerinic 3, Lesley Saketkoo 4, Kristin Highland5, Hilary Wilson 6, Margarida Alves 7, Nils Schoof 6 and Toby Maher 8, 1Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Germany, Germany, Germany, 2Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 3University of Florence, Department of Medicine, Florence, Italy, Florence, Italy, 4New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, 5Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, 6Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, 7Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, 8National Heart and Lung Institute, Imperial College London, UK and National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, London, United Kingdom

    Background/Purpose: SSc-ILD is a major cause of morbidity and mortality in patients with systemic sclerosis. A subset of patients with SSc-ILD show a decline in…
  • Abstract Number: 1657 • 2019 ACR/ARP Annual Meeting

    Sarcopenia in Systemic Sclerosis

    Iván Dario Remolina Rincón1, Maria Alak 2, Graciela Alsina 3, Pedro Quevedo 2, Mariano Rivero 4 and Damian Duartes 5, 1Hospital Británico, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina, 2Hospital Britanico de Buenos Aires, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina, 3Consultorios Pampa, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina, 4Hospital Británico, Buenos Aires, Buenos Aires, Argentina, 5Hospital Británico, Ciudad Autonoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina

    Background/Purpose: Gastrointestinal tract involvement in systemic sclerosis (SSc) occurs in almost all patients varying in severity and affecting motility, digestion, absorption and excretion. These abnormalities…
  • Abstract Number: 1658 • 2019 ACR/ARP Annual Meeting

    Impact of Visceral Involvement in the Characterization and Prognosis of Patients Without Skin Involvement Classified as Systemic Sclerosis (SSc) According to 2013 ACR/EULAR Criteria in a Large Single Cohort

    Ana Ribeiro1, Ana Paula Luppino-Assad 2, Henrique Silva 3, Leandro Prado 3, Danieli Andrade 4 and Percival Degrava Sampaio-Barros 5, 1HOSPITAL DAS CLINICAS DA FACULDADE DE MEDICINA DE SAO PAULO, Sao Paulo, Brazil, 2Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil., Sao Paulo, Brazil, 3HOSPITAL DAS CLINICAS DA FACULDADE DE MEDICINA DA UNIVERSIDADE DE SAO PAULO, Sao Paulo, Brazil, 4Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil, 5Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR, Brazil, Sao Paulo, Brazil

    Background/Purpose: The 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) allowed the inclusion of a subset of patients without skin involvement, not considered by the…
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