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  • Abstract Number: 2594 • 2019 ACR/ARP Annual Meeting

    Associations Between Antibodies to the Angiotensin II Type 1 Receptor and Endothelian-1 Type a Receptor and the Incidence of Vascular Complications in Early Diffuse Systemic Sclerosis

    Kendall Downer1, Robyn Domsic 2, Carol Bentlejewski 2, Adriana Zeevi 2 and Lei Zhu 2, 1University of Pittsburgh Medical Center, Pittsburgh, PA, 2University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Recently, the hypothesis that autoantibodies to the angiotensin II type 1 (AT1R) and endothelian-1 type A (ETAR) receptors are driving the vascular injury and…
  • Abstract Number: 2595 • 2019 ACR/ARP Annual Meeting

    Asymptomatic Scleroderma Antibody Positivity and Progression to Systemic Sclerosis

    Derek Jones 1, Marissa Mangini 1, Sydney Wearing 1 and Victoria Shanmugam2, 1The George Washington University School of Medicine and Health Sciences, Washington, DC, 2George Washington University, Georgetown, DC

    Background/Purpose: Systemic sclerosis is an autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin, vasculature and internal organs along with disease specific autoantibody…
  • Abstract Number: 2596 • 2019 ACR/ARP Annual Meeting

    The Contribution of Left Heart Disease in Patients with Systemic Sclerosis-associated Pulmonary Hypertension Having Normal Pulmonary Artery Wedge Pressure

    Yoshioki Yamasaki1, Kanako Suzuki 1, Keiichi Sakurai 2, Yusa Asari 1, Hidehiro Yamada 3 and Kimito Kawahata 2, 1Rheumatology and Allergology, St. Marianna University School of Medicine, Kawasaki, Japan, 2Rheumatology and Allergology, St. Marianna University School of Medicine, Tokyo, Japan, 3Center for Rheumatic diseases, Seirei Yokohama Hospital, Kawasaki, Japan

    Background/Purpose: Patients with systemic sclerosis (SSc) complicates variety of cardiopulmonary diseases which can result in pulmonary hypertension (PH). The types of PH are usually classified…
  • Abstract Number: 2597 • 2019 ACR/ARP Annual Meeting

    Prevalence and Clinical Associations of Degos Lesions in Systemic Sclerosis

    Paula Song1, Melody Chung 2, Shufeng Li 3, David Fiorentino 4 and Lorinda Chung 2, 1Department of Medicine, Santa Clara Valley Medical Center, Palo Alto, CA, 2Stanford University, Palo Alto, CA, 3Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA, 4Department of Dermatology, Stanford University School of Medicine, Redwood City, CA

    Background/Purpose: Degos disease, also known as malignant atrophic papulosis (MAP), is a rare small-vessel vasculopathy that has characteristic cutaneous manifestations consisting of erythematous telangiectatic rims…
  • Abstract Number: 2598 • 2019 ACR/ARP Annual Meeting

    Different Treatment Backgrounds Do Not Influence Aminaphtone Efficacy in Primary and Secondary Raynaud’s Phenomenon

    Alberto Sulli1, Federica Goegan 2, Emanuele Gotelli 2, Massimo Patane' 3, Carmen Pizzorni 3, Sabrina Paolino 3, Elisa Alessandri 4 and Barbara Ruaro 5, 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genoa, Italy, 2Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genova, Italy., Genoa, Italy, 3Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 4Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genova, 51 Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy; 2 Cardinal Ferrari Centre, S. Stefano Rheabilitation, Fontanellato (Parma), Italy, Genoa, Italy

    Background/Purpose: Aminaphtone treatment improves clinical symptoms of Raynaud's phenomenon (RP) and increases peripheral blood perfusion (BP) in patients with either primary or secondary RP (1-2).The…
  • Abstract Number: 2599 • 2019 ACR/ARP Annual Meeting

    Body Composition and Nailfold Videocapillaroscopy Patterns in a Cohort of Systemic Sclerosis Patients

    Tomatis Veronica 1, Sabrina Paolino 2, Alberto Sulli3, Emanuele Gotelli 4, Andrea Casabella 5, Francesco Cattelan 6, Schenone Carlotta 1, Carmen Pizzorni 2, Elisa Alessandri 7 and Maurizio Cutolo 8, 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genova, Liguria, Italy, 2Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 3Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genoa, Italy, 4Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genova, Italy., Genoa, Italy, 51 Research Laboratory and Academic Division of the Clinical Rheumatology Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 6Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy, 7Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genova, 8Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genoa, Italy

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disease, characterized by microvascular damage and progressive fibrosis. Among clinical complications, abnormal body composition and sarcopenia have been reported…
  • Abstract Number: 2600 • 2019 ACR/ARP Annual Meeting

    ‘If You Don’t Use It, You Lose It’: Rehabilitation of Finger Dexterity and Ability to Perform Activities of Daily Living in Systemic Sclerosis

    Elena Eusterwiemann1, Marina Anderson 2, Mark Robinson 1 and Gabor Barton 1, 1Liverpool John Moores University, Liverpool, United Kingdom, 2Aintree University Hospital, Liverpool

    Background/Purpose: Hand involvement due to increased skin thickness and skin collagen content is one of the first manifestations of systemic sclerosis (SSc) leading to a…
  • Abstract Number: 2601 • 2019 ACR/ARP Annual Meeting

    Trabecular Bone Score and Malnutrition in a Cohort of Systemic Sclerosis Patients

    Massimo Patane' 1, Sabrina Paolino 1, Veronica Tomatis 2, Andrea Casabella 3, Carmen Pizzorni 1, Luca Carmisciano 4, Alessio Signori 4 and Maurizio Cutolo5, 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 2Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.2Biostatistics Unit, Department of Health Sciences, University of Genoa, Genoa, Italy, genova, Liguria, Italy, 31 Research Laboratory and Academic Division of the Clinical Rheumatology Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 4Biostatistics Unit, Department of Health Sciences, University of Genoa, Genoa, Italy, 5Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genoa, Italy

    Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease characterized by microvascular damage, immune system activation and progressive fibrosis of the skin and internal organs.…
  • Abstract Number: 2602 • 2019 ACR/ARP Annual Meeting

    Changes in Fecal Microbiota Composition After Fecal Microbiota Transplantation in Systemic Sclerosis

    Anna Maria Hoffmann-Vold1, Håvard Fretheim 2, Brian Chung 3, Henriette Didriksen 3, Espen Bækkevold 3, Øyvind Midtvedt 3, Cathrine Brunborg 3, Kristian Holm 3, Anders Tennøe 3, Torhild Garen 3, Tore Midtvedt 4, Marius Trøseid 3, Johannes Hov 3, Knut Lundin 3 and Øyvind Molberg 2, 1Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 2University Hospital Oslo, Oslo, Norway, 3Oslo University Hospital, Oslo, Norway, 4Karolinska University Hospital, Stockholm, Sweden

    Background/Purpose: In the double-blind, placebo-controlled 16-week pilot including 10 systemic sclerosis (SSc) patients with upper and lower gastrointestinal (GI) symptoms we found that fecal microbiota…
  • Abstract Number: 2603 • 2019 ACR/ARP Annual Meeting

    Course of Progressive Lung Fibrosis in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) in the EUSTAR Database

    Anna-Maria Hoffmann-Vold1, Yannick Allanore 2, Margarida Alves 3, Nicole Graf 4, Paolo Airò 5, Lidia P. Ananyeva 6, László Czirják 7, Serena Guiducci 8, Eric Hachulla 9, Mengtao Li 10, Carina Mihai 11, Gabriela Riemekasten 12, Petros Sfikakis 13, Gabriele Valentini 14, Otylia Kowal-Bielecka 15 and Oliver Distler 16, 1Dept. of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 2Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, 3Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, 4Graf Biostatistics, Winterthur, Switzerland, Winterthur, Switzerland, 5UO Reumatologia e Immunologia Clinica, Spedali Civili di Brescia, Brescia, Italy, Brescia, Italy, 6VA Nasonova Institute of Rheumatology, Moscow, Russian Federation, Moscow, Russia, 7Dept. of Rheumatology and Immunology, University of Pécs, Pécs, Hungary, Pécs, Hungary, 8Dept. of Clinical and Experimental Medicine, Section of Rheumatology, University of Florence, Italy, Florence, Italy, 9Dept. of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, University of Lille, Lille, France, Lille, France, 10Dept. of Rheumatology, Peking Union Medical College Hospital (West Campus), Beijing, China, Beijing, China (People's Republic), 11Dept. of Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, 12Dept. of Rheumatology and Clinical Immunology, University Medical Center Schleswig-Holstein, Lübeck, Germany, Lübeck, Germany, 13Joint Rheumatology Programme, National & Kapodistrian University of Athens Medical School, Athens, Greece, Athens, Greece, 14Dipartimento di Medicina di Precisione, II Policlinico U.O. Reumatologia, Napoli, Italy, Napoli, Italy, 15Dept. of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, Bialystok, Poland, 16Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland

    Background/Purpose: The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is heterogeneous; some patients may experience rapid decline in lung function, while others have relatively…
  • Abstract Number: 2604 • 2019 ACR/ARP Annual Meeting

    Pain Chronification and the Important Role of Non-disease Specific Symptoms in Patients with Systemic Sclerosis

    Caroline Evers 1, Suzana Jordan 1, Britta Maurer 1, Rucsandra Dobrota 1, Petra Hoederath 2 and Oliver Distler3, 1Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, 2Centre for Neurosurgery, Hirslanden, St. Gallen, St. Gallen, Switzerland, 3Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland

    Background/Purpose: Pain is a frequent, yet inadequately explored challenge in patients with systemic sclerosis (SSc). The aim of this study was to conduct an extensive…
  • Abstract Number: 2605 • 2019 ACR/ARP Annual Meeting

    Men and Black Persons Die at Younger Ages from Systemic Sclerosis: A Nationwide Population-based Study

    Ram Raj Singh1, Devanshu Singh 2 and Eric Yen 1, 1UCLA, Los Angeles, 2Johns Hopkins University, Baltimore, MD

    Background/Purpose: Systemic sclerosis (SSc) is more common in women than men, but men tend to have a faster disease progression than women. However, conflicting results…
  • Abstract Number: 2606 • 2019 ACR/ARP Annual Meeting

    Evolution of Systemic Sclerosis-Related Interstitial Lung Disease After Autologous Hematopoietic Stem Cell Transplantation

    Jacopo Ciaffi1, Nina van Leeuwen 2, Maaike Boonstra 2, Lucia Kroft 3, Anne Schouffoer 3, Maarten Ninaber 2, Thomas Huizinga 2 and Jeska de Vries-Bouwstra 2, 1Leiden University Medical Center, Bologna, Emilia-Romagna, Italy, 2Leiden University Medical Center, Leiden, Netherlands, 3Leiden University Medical Centre, Leiden, Zuid-Holland, Netherlands

    Background/Purpose: Three trials demonstrated the overall superiority of autologous hematopoietic stem cell transplantation (HSCT) compared to pulsed cyclophosphamide (CYC) in SSc. An improvement in forced…
  • Abstract Number: 2607 • 2019 ACR/ARP Annual Meeting

    Ultrasound Detection of Calcinosis and Correlation with Ulnar Artery Occlusion in Patients with Systemic Sclerosis

    Robert Fairchild1, Melody Chung 1, Laurel Sharpless 1, Shufeng Li 2 and Lorinda Chung 1, 1Stanford University, Palo Alto, CA, 2Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA

    Background/Purpose: Systemic Sclerosis (SSc) is a progressive fibrotic and vascular disease affecting multiple organs. Calcinosis cutis in SSc is characterized by calcium deposition in the…
  • Abstract Number: 2608 • 2019 ACR/ARP Annual Meeting

    Subsets in Systemic Sclerosis-ILD: Working Towards Consensus-Based Definitions

    David Roofeh1, Kevin Brown 2, Donald Tashkin 3, Shervin Assassi 4, Shaney Barratt 5, Elana Bernstein 6, Nitin Bhatt 7, Lorinda Chung 8, Harold Collard 9, Richard Conway 10, Paul Dellaripa 11, Christopher Denton 12, Oliver Distler 13, Robyn Domsic 14, Tracy J. Doyle 15, Puneet Garcha 16, Nishant Gupta 17, Anna Maria Hoffmann-Vold 18, Vivien Hsu 19, Francesca Ingegnoli 20, Kerri Johannson 21, Bashar Kahaleh 22, Leticia Kawano-Dourado 23, Ella Kazerooni 1, Surabhi Khanna 17, Peter Korsten 24, Celia JF Lin 25, Toby Maher 26, Stephen Mathai 27, Fernando Martinez 28, Eric Matteson 29, Vivek Nagaraja 30, Chester Oddis 31, Juan Ovalles-Bonilla 32, John Pauling 33, Ganesh Raghu 34, Ignasi Rodriguez-Pinto 35, Ivan Rosas 15, James Seibold 36, Virginia Steen 37, Vibeke Strand 38, Elizabeth Volkmann 39, Dharshan Vummidi 30, Simon Walsh 40, Donald Zoz 41, Athol Wells 42 and Dinesh Khanna 43, 1University of Michigan, Ann Arbor, 2National Jewish Health, Denver, 3University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, 4Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, 5North Bristol NHS Trust, Bristol, England, United Kingdom, 6Columbia University, New York City, 7Ohio State University Wexner Medical Center, Columbus, 8Stanford University, Palo Alto, CA, 9University of California San Fransisco, San Fransisco, 10Blackrock Clinic, Dublin, Ireland, 11Brigham and Women's Hospital, Boston, MA, 12University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, 13Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, 14University of Pittsburgh, Pittsburgh, PA, 15Brigham and Women's Hospital, Harvard Medical School, Boston, 16Baylor Scott & White Health, Dallas, 17University of Cincinnati, Cincinnati, 18Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 19Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ, 20Division of Clinical Rheumatology, ASST Pini-CTO, Università degli Studi di Milano, Department of Clinical Sciences and Community Health, Milan, Italy, Milano, Italy, 21University of Calgary, Calgary, 22University of Toledo, Toledo, OH, 23University of Sao Paulo, Sao Paulo, Brazil, 24University Medical Center Göttingen, Göttingen, Germany, 25Genentech, San Fransisco, 26National 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, 27John Hopkins Pulmonary and Critical Care Medicine, Baltimore, 28Will Cornell Medicine Pulmonary & Critical Care Medicine, New York, 29Mayo Clinic College of Medicine and Science, Rochester, Minnesota, USA, Rochester, 30University of Michigan, Ann Arbor, MI, 31University of Pittsburgh Medical Center, Pittsburgh, PA, 32Hospital Gregorio Marañón, Madrid, Spain, 33Department of Rheumatology, Royal National Hospital of Rheumatic Diseases, Royal United Hospitals NHS Foundation Trusts, Bath, England, United Kingdom, 34University of Washington, Seattle, USA, Seattle, 35University of Barcelona, Barcelona, Spain, 36Scleroderma Research Consultants LLC, Aiken, South Carolina, USA, Aiken, 37Georgetown University, Washington, D.C., USA, Georgetown, 38Division of Immunology/Rheumatology, Stanford University, Stanford, CA, 39University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, 40Kings College London, London, United Kingdom, 41Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, Connecticut, USA, Ridgefield, CT, 42Royal Bromptom Hospital, London, United Kingdom, 43Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor

    Background/Purpose: Systemic sclerosis associated interstitial lung disease (SSc-ILD) is heterogeneous, with varying degrees of severity and risk of disease progression.  No consensus-based definitions of disease…
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