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  • Abstract Number: 786 • 2018 ACR/ARHP Annual Meeting

    Comparison of Nailfold Microvascular Damage in Mixed Connective Tissue Disease Versus Systemic Sclerosis Patients during a Three Year Follow-up

    Alberto Sulli1, Giorgia Ferrari1, Carmen Pizzorni1, Barbara Ruaro1, Sabrina Paolino2, Vanessa Smith3 and Maurizio Cutolo1, 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, San Martino Polyclinic Hospital, Genoa, Italy, Genoa, Italy, 2Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, San Martino Polyclinic Hospital, Genoa, Italy, Genova, Italy, 3Department of Internal Medicine, Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, Ghent, Belgium, Ghent, Belgium

    Background/Purpose: In systemic sclerosis (SSc), capillary abnormalities progress in a clearly defined sequence called the "scleroderma patterns" (Early, Active, Late) (1-3). On the contrary, characteristic…
  • Abstract Number: 787 • 2018 ACR/ARHP Annual Meeting

    Longitudinal Follow-up of Anti-Topoisomerase I Positive Patients within the Leiden Systemic Sclerosis Cohort – Prognosis Infaust?

    Maaike Boonstra1, Maarten K. Ninaber2, Nina Ajmone Marsan3, Hans U. Scherer1, Tom W.J. Huizinga1 and Jeska de Vries-Bouwstra1, 1Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 2Heart and Lung Center; Pulmonology, Leiden University Medical Center, Leiden, Netherlands, 3Heart and Lung Center, Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: SSc is known for its heterogeneous disease course in which anti-topoisomerase I antibodies (ATA) are associated with dcSSc and interstitial lung disease and therefore…
  • Abstract Number: 788 • 2018 ACR/ARHP Annual Meeting

    Clinical and Echocardiographic Associates of All-Cause Mortality and Cardiovascular Outcomes in Patients with Systemic Sclerosis

    Susanne Van Wijngaarden1, Maaike Boonstra2, Bas Bloem3, Daniela Cassani4, Felix Tanner4, Suzana Jordan5, Oliver Distler6, Martin Jan Schalij3, V. Delgado1, J.J. Bax1, Jeska de Vries-Bouwstra7 and Nina Ajmone Marsan1, 1Heart and Lung Center, Leiden University Medical Center, Leiden, Netherlands, 2Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 3Leiden University Medical Center, Leiden, Netherlands, 4University Heart Center, University Hospital Zurich, Zurich, Switzerland, 5Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 6Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 7Rheumatology, Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: Cardiac events are an important cause of mortality in Systemic Sclerosis (SSc), but its diagnosis remains challenging. Left ventricular global longitudinal strain is a…
  • Abstract Number: 789 • 2018 ACR/ARHP Annual Meeting

    The Relationship between YKL-40 and Vascular Endothelial Growth Factor in Angiogenesis in Systemic Sclerosis Patients

    Tetsuya Furukawa1, Kiyoshi Matsui2, Masayasu Kitano2 and Naoto Azuma2, 1Division ofRheumatology Department of internal medicine, Hyogo College of Medicine, Nishinomiya, Japan, 2Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Japan

    Background/Purpose: Systemic sclerosis (SSc) is an intractable, connective tissue disease that causes fibrosis of the skin and organs and its prognosis is affected by pulmonary…
  • Abstract Number: 790 • 2018 ACR/ARHP Annual Meeting

    Gastrointestinal Symptom Burden and Quality of Life in Systemic Sclerosis: Understanding the Role of Diet

    Kelly Jensen1, Linda Wang2, Rosemary Kovacic2, Veronika K. Jaeger3, Monika Lammi4 and Lesley Ann Saketkoo5, 1Tulane University School of Medicine, New Orleans, LA, 2Tulane University School of Public Health and Tropical Medicine, New Orleans, LA, 3Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 4GI Motility Program at Ochsner Medical Center, New Orleans, LA, 5Tulane, New Orleans, LA

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disease affecting multiple organ systems including vascular, musculoskeletal, cardiac, renal, pulmonary, and gastrointestinal (GI). 90% of SSc…
  • Abstract Number: 791 • 2018 ACR/ARHP Annual Meeting

    Safety and Suitability of a Direct Thrombin Inhibitor, Dabigatran Etexilate, in Scleroderma-Associated Interstitial Lung Disease (SSc-ILD) Patients

    Rick Silver1, Ilia Atanelishvili2, Tanjina Akter2, Kelley Kajdasz3, Dulaney Wilson4, Paul J. Nietert5, J. Terrill Huggins6, Kristin B. Highland7 and Galina S. Bogatkevich2, 1Rheumatology, Medical University of SC, Charleston, SC, 2Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, 3Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, 4Public Health Sciences, Medical University of South Carolina, Charleston, SC, 5Public Health Science, Medical University of South Carolina, Charleston, SC, 6Pulmonology, Medical University of South Carolina, Charleston, SC, 7Rheumatology.org, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Studies from our laboratory and others have shown thrombin to be a fibrogenic mediator implicated in the pathogenesis of ILD, including scleroderma-associated ILD (SSc-ILD).…
  • Abstract Number: 792 • 2018 ACR/ARHP Annual Meeting

    Evaluation of Esophageal Dysmotility in Systemic Sclerosis: Clinical VALUE of Computed Tomography

    Cristina Sobrino1, Cristina Pijoan-Moratalla1, Nicolás Almeida-Arostegui2, Luis Gorospe-Sarasúa2 and Carlos de la Puente-Bujidos1, 1Rheumatology Unit. Ramón y Cajal University Hospital, Madrid, Spain, 2Radiology Unit. Ramón y Cajal University Hospital, Madrid, Spain

    Background/Purpose: Esophageal dysmotility is common in Systemic Sclerosis (SSc), affecting 50-80% of patients, usually associated with poor prognosis. SSc leads to atrophy and fibrosis of…
  • Abstract Number: 793 • 2018 ACR/ARHP Annual Meeting

    Clinical Associations of Anti-U11/U12 (RNPC-3) Autoantibodies in Patients with Systemic Sclerosis

    Lorenzo Beretta1, Michael Mahler2, Chelsea Bentow2, Andrea Seaman2, Fabrece Roup2, Michelle Amio2, Karl Norvell2, Jay Milo2, Susan Encabo2, Janire Perurena3, Maite Sanz3, Alfredo Guillen4, Ana Marín3, Vicent Fonollosa3, Eduardo Callejas3 and Carmen Pilar Simeón4, 1Scleroderma Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy, 2Research and Development, Inova Diagnostics, San Diego, CA, 3Scleroderma Unit, Internal Medicine Department, Vall d’Hebron Hospital, Barcelona, Spain, 4Scleroderma Unit, Internal Medicine Department, Vall d’Hebron Hospital, Barcelona, Spain

    Background/Purpose: Anti-nuclear antibodies (ANA) are present in approximately 90% of sera from systemic sclerosis (SSc) patients and play an important role in the diagnosis and…
  • Abstract Number: 794 • 2018 ACR/ARHP Annual Meeting

    Identification of an Immunodominant Epitope on Rnpc-3 As a Target of Autoantibodies in Patients with Systemic Sclerosis

    Michael Mahler1, Chelsea Bentow1, Jay Milo1, Marie Hudson2,3, Murray Baron3, May Choi4 and Marvin J. Fritzler4, 1Research and Development, Inova Diagnostics, San Diego, CA, 2Division of Rheumatology, Jewish General Hospital, Lady Davis Institute for Medical Research, Montreal, QC, Canada, 3Department of Medicine, McGill University, Montreal, QC, Canada, 4Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

    Background/Purpose: Anti-nuclear antibodies (ANA) are present in approximately 90% of sera from systemic sclerosis (SSc) patients and play an important role in the diagnosis and…
  • Abstract Number: 795 • 2018 ACR/ARHP Annual Meeting

    Frequency and Clinical Associations of Rare Antibodies in a Large Connective Tissue Disease Cohort

    Kristina E.N. Clark1, Corrado Campochiaro2, Lauren V. Host3, Alper Sari4, Svetlana I. Nihtyanova5, Carmen Fonseca6, Christopher P. Denton7 and Voon H. Ong8, 1Centre of Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 2Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, London, United Kingdom, 3Department of Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 4Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, 5Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 6UCL Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, 7University College London, London, United Kingdom, 8Division of Medicine, University College London, London, United Kingdom

    Background/Purpose: Connective tissue diseases (CTDs) are characterised by specific autoantibodies which are useful for diagnosis. Rare antibodies have been described in CTDs in several case…
  • Abstract Number: 796 • 2018 ACR/ARHP Annual Meeting

    Demographic and Clinical Features of Systemic Sclerosis Patients with Anti-U1RNP Antibodies: A European Scleroderma Trials and Research (EUSTAR) Analysis

    Wanlong Wu1,2, Petra Hoederath1, Eric Hachulla3, Paolo Airò4, Gabriele Valentini5, Marco Matucci Cerinic6, Franco Cozzi7, Gabriela Riemekasten8, Yannick Allanore9, Patricia Carreira10, Suzana Jordan11 and Oliver Distler1, 1Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Department of Rheumatology, South Campus, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China, 3Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, University of Lille, Lille, France, 4Rheumatology and Clinical immunology Unit, Spedali Civili of Brescia, Brescia, Italy, 5Department of Clinical and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, 6Division of Rheumatology, Division of Rheumatology, University of Florence, Florence, Italy, 7Division of Rheumatology, Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy, 8Rheumatology, University Hospital Schleswig-Holstein - Campus Lübeck, Lübeck, Germany, 9Cochin Hospital, Paris Descartes University, Paris, France, Paris, France, 10Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain, 11Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: Anti-U1RNP antibodies can be detected in patients with systemic sclerosis (SSc). However, their prevalence and clinical correlation with organ-specific complications have not been well…
  • Abstract Number: 797 • 2018 ACR/ARHP Annual Meeting

    Absolute Reduction of Peripheral CD4+ Regulatory T Cells in Patients with Systemic Sclerosis and Its Restoration By Short-Term and Low Dose IL-2 Treatment

    Lili Shang1, Jing Luo2, Chong Gao3, Jingman Yuan1, Qian Li1, Xiaoli Liu1, Huiying Gao1 and Xiao-Feng Li4, 1Department of Rheumatology, The Second Hospital of Shanxi Medical University, Taiyuan, China, 2the Second Hospital of Shanxi Medical University, Taiyuan, China, 3Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, Cambridge, MA, 4Rheumatology, the Second Hospital of Shanxi Medical University, Taiyuan, China

    Background/Purpose: The aim of the present study was to investigate whether the imbalance of CD4+ T subsets can be corrected by supplementing low dose interleukin -2 (IL-2).Methods:…
  • Abstract Number: 798 • 2018 ACR/ARHP Annual Meeting

    Predominant Fasciitis and Mild Intramuscular Edema on Muscle Magnetic Resonance Imaging in Scleroderma-Associated Myopathy

    Julie J. Paik1, Shivani Ahlawat2, Filippo DelGrande3, Laura K. Hummers4, Fredrick M. Wigley4, Ami A. Shah4, Andrew Mammen5 and Laura Fayad6, 1Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 2Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, 3Johns Hopkins University, Baltimore, MD, 4Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 5National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 6Radiology, Johns Hopkins University, Baltimore, MD

    Background/Purpose: Skeletal myopathy in systemic sclerosis is poorly defined. The spectrum of muscle histopathology in systemic sclerosis has been previously studied and shown to be…
  • Abstract Number: 799 • 2018 ACR/ARHP Annual Meeting

    Progressive Lung Fibrosis in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease in the Eustar Database

    Anna-Maria Hoffmann-Vold1, Yannick Allanore2, Margarida Alves3, Nicole Graf4, Paolo Airò5, Lidia Ananyeva6, László Czirják7, Serena Guiducci8, Eric Hachulla9, Mengtao Li10, Carina Mihai11, Petros Sfikakis12, Gabriele Valentini13, Otylia Kowal-Bielecka14 and Oliver Distler11, 1Oslo University Hospital, Oslo, Norway, 2Service de Rhumatologie A, Hôpital Cochin, Paris, France, 3Boehringer Ingelheim International GmbH, Ingelheim, Germany, 4Graf Biostatistics, Winterthur, Switzerland, 5UO Reumatologia e Immunologia Clinica, Spedali Civili di Brescia, Brescia, Italy, 6VA Nasonova Institute of Rheumatology, Moscow, Russian Federation, 7Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary, 8Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi di Firenze, Firenze, Italy, 9Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, University of Lille, Lille, France, 10Department of Rheumatology, Peking Union Medical College Hospital (West Campus), Beijing, China, 11Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 12Joint Rheumatology Programme, National & Kapodistrian University of Athens Medical School, Athens, Greece, 13Dipartimento di Medicina di Precisione, II Policlinico U.O. Reumatologia, Napoli, Italy, 14Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland

    Background/Purpose: Systemic sclerosis (SSc) carries a high risk for interstitial lung disease (ILD). Patients with SSc-ILD are prone to develop progressive lung fibrosis, but there…
  • Abstract Number: 800 • 2018 ACR/ARHP Annual Meeting

    Assessment of Recent Evidence to Support Treatment Recommendations in Patients with SSc-ILD

    Anna-Maria Hoffmann-Vold1, Toby Maher2, Edward Philpot3, Ali Ashrafzadeh4, Diwakar Jha5, Margarida Alves6 and Oliver Distler7, 1Oslo University Hospital, Oslo, Norway, 2Royal Brompton Hospital, London, United Kingdom, 3IQVIA, Durham, NC, 4IQVIA, Los Angeles, CA, 5IQVIA, Gurugram, India, 6Boehringer Ingelheim International GmbH, Ingelheim, Germany, 7Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: Systemic Sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of skin and internal organs with an estimated worldwide prevalence of 110-430 cases/million.…
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