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  • Abstract Number: 727 • 2017 ACR/ARHP Annual Meeting

    Survival and Clinical-Capillaroscopic Characteristics of French Canadian Systemic Sclerosis Patients: Analysis Based on Systemic Sclerosis Autoantibodies and the Novel Anti-BICD2 Autoantibody

    Boyang Zheng1, Michael Mahler2, Jean-Luc Senécal3, France Joyal4 and Martial Koenig5, 1Division of Internal Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada, 2Research and Development, Inova Diagnostics, San Diego, CA, 3Rheumatology, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, QC, Canada, 4Internal Medicine, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada, 5Internal Medicine, Hôpital Notre-Dame du CHUM, Montréal, QC, Canada

    Background/Purpose: Systemic sclerosis (SSc) autoantibodies (aAbs) are invaluable for SSc diagnosis and prognosis. Anti-centromere (ACA), anti-topoisomerase I (ATA) and anti-RNA polymerase III (RNAP) have been…
  • Abstract Number: 728 • 2017 ACR/ARHP Annual Meeting

    Symptoms of Autonomic Dysfunction in Systemic Sclerosis Assessed By the Compass-31 Questionnaire

    Brittany Adler1, James Russell2, Laura K. Hummers3 and Zsuzsanna McMahan4, 1Rheumatology, Johns Hopkins University, Baltimore, MD, 2Neurology, Anatomy and Neurobiology, University of Maryland School of Medicine, Baltimore, MD, 3Medical and Rheumatology, Johns Hopkins University, Baltimore, MD, 4Department of Internal Medicine, Johns Hopkins University, Baltimore, MD

    Background/Purpose: Autonomic dysfunction is a known complication of systemic sclerosis (SSc) and can affect vascular tone, gastrointestinal (GI) motility, and heart rate and blood pressure…
  • Abstract Number: 729 • 2017 ACR/ARHP Annual Meeting

    Clinically Relevant Serum Proteins in Patients with Early Diffuse Cutaneous Systemic Sclerosis

    Guoshuai Cai1, Kelsey S. Flood2, Shervin Assassi3, Elana J. Bernstein4, Robyn T. Domsic5, Jessica K. Gordon6, Faye Hant7, Elena Schiopu8, Virginia D. Steen9, Tracy M. Frech10, Dinesh Khanna11, Ami A. Shah12, Victoria K. Shanmugam13, Flavia V. Castelino14 and Monique Hinchcliff15, 1Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 2Internal Medicine, Northwestern Medicine, Chicago, IL, 3University of Texas McGovern Medical School, Houston, TX, 4Rheumatology, Columbia University, New York, NY, 5Rheumatology, University of Pittsburgh, Pittsburgh, PA, 6Rheumatology, Hospital for Special Surgery, New York, NY, 7Medicine/Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, 8Internal Medicine, University of Michigan Medical System, Ann Arbor, MI, 9Rheumatology, MedStar Georgetown University Hospital, Washington, DC, 10Division of Rheumatology, University of Utah, Salt Lake City, UT, 11Division of Rheumatology and Clinical Autoimmune Center of Excellence, University of Michigan, Ann Arbor, MI, Ann Arbor, MI, 12Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 13Rheumatology, The George Washington University, Washington, DC, 14Rheumatology, Harvard Medical School, Boston, MA, 15Rheumatology, Northwestern Medicine, Chicago, IL

    Background/Purpose: The Prospective Registry of Early Systemic Sclerosis (PRESS), an 11 center US cohort study of early diffuse cutaneous systemic sclerosis (dcSSc) patients, was designed…
  • Abstract Number: 730 • 2017 ACR/ARHP Annual Meeting

    The Association of Pulmonary Hypertension with Isolated Nucleolar Serum Autoantibodies in Systemic Sclerosis

    Kathleen Aren1, Mary A. Carns1, Michael Cuttica2, Julia (Jungwha) Lee3, Virginia D. Steen4 and Monique Hinchcliff5, 1Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 2Northwestern University, Feinberg School of Medicine, Division of Pulmonary and Critical Care, Chicago, IL, 3Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, 4Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, 5Department of Medicine Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL

    Background/Purpose:  Serum antibodies are used to identify SSc patients who may be at higher risk for SSc-PH. The Pulmonary Hypertension Assessment and Recognition of Outcomes…
  • Abstract Number: 731 • 2017 ACR/ARHP Annual Meeting

    Prediction of Progression of Interstitial Lung Disease in Patients with Systemic Sclerosis

    Wanlong Wu1, Suzana Jordan2, Mike Oliver Becker3, Rucsandra Dobrota3, Shuang Ye4, Britta Maurer5 and Oliver Distler2, 1Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich; Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiao Tong University School of Medicine, Zurich, Switzerland, 2Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 3Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 4Department of Rheumatology, Renji Hospital South Campus, School of Medicine, Shanghai Jiao Tong University, Shanghai, China, 5Department of Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: No data are available to distinguish between progressive and stable patients when mild lung fibrosis is diagnosed in systemic sclerosis (SSc) patients. This study…
  • Abstract Number: 732 • 2017 ACR/ARHP Annual Meeting

    Progression of Skin Fibrosis Is Associated with Decline in Lung Function in Patients with Diffuse Cutaneous Systemic Sclerosis: A European Scleroderma Trials and Research (EUSTAR) Analysis

    Wanlong Wu1, Suzana Jordan2, Nicole Graf3, Janethe Pena4, John Curram5, Yannick Allanore6, Marco Matucci-Cerinic7, Janet E. Pope8, Christopher Denton9, Dinesh Khanna10 and Oliver Distler1, 1Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 3Graf Biostatistics, Winterthur, Switzerland, 4Bayer HealthCare Pharmaceuticals Inc, Whippany, NJ, 5Bayer Plc, Newbury, United Kingdom, 6Department of Rheumatology, Cochin Hospital, Paris Descartes University, Paris, France, 7Dept of Medicine/Div of Rheum, University of Florence, Florence, Italy, 8Department of Medicine, Division of Rheumatology, University of Western Ontario, St Joseph's Health Care, London, ON, Canada, 9Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom, 10Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI

    Background/Purpose: Previously, we have identified short disease duration (≤15 months) and low baseline modified Rodnan skin score (mRSS) (≤22/51) as independent predictors of progressive skin…
  • Abstract Number: 733 • 2017 ACR/ARHP Annual Meeting

    Ethnic Variation in Systemic Sclerosis Morbidity and Mortality

    Sindhu Johnson1, Zareen Ahmad2 and Haifa Al Sheikh3, 1Division of Rheumatology, Toronto Western Hospital, Mount Sinai Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada, 2Toronto Scleroderma Program, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 3Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada

    Background/Purpose: Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by pathological skin thickening and can involve multiple internal organs. Ethnic variations in SSc…
  • Abstract Number: 734 • 2017 ACR/ARHP Annual Meeting

    Correlation of the American College of Rheumatology Provisional Composite Response Index in Systemic Sclerosis with Serum Biomarkers of Fibrogenesis in an Observational Cohort

    Giuseppina Abignano1, Sookhoe Eng2, Maya H. Buch3, Paul Emery4, Dinesh Khanna5 and Francesco Del Galdo2, 1Rheumatology Department of Lucania,, Rheumatology Institute of Lucania (IReL), San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera,, Potenza, Italy, 2Leeds Musculoskeletal Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 3NIHR-Leeds Musculoskeletal Biomedical Research Unit, Leeds, United Kingdom, 4NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 5University of Michigan, Ann Arbor, MI

    Background/Purpose: The CRISS is a composite index of response in diffuse cutaneous systemic sclerosis (dcSSC). It is a 2-step process for the probability of improvement…
  • Abstract Number: 735 • 2017 ACR/ARHP Annual Meeting

    Cardiopulmonary Exercise Testing to “Detect” Pulmonary Arterial Hypertension in Systemic Sclerosis

    Rosa Casella1, Alessandro Santaniello2,3, Marco Vicenzi1 and Lorenzo Beretta2, 1Cardiovascular Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, 2Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 3VIA FRANCESCO SFORZA 28, OSPEDALE MAGGIORE POLICLINICO, MILANO, Italy

    Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading death causes in SSc patients. To gain a better prognosis, screening methods are needed to…
  • Abstract Number: 736 • 2017 ACR/ARHP Annual Meeting

    N-Terminal Pro-Brain Natriuretic Peptide Is Disproportionately Elevated in Scleroderma Associated Pulmonary Arterial Hypertension Compared to Idiopathic Pulmonary Arterial Hypertension

    Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1,7, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, 3Rheumatology.org, Cleveland Clinic, Cleveland, OH, 4Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, 5Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, 6Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, 7Rheumatic and Immunologic Ds, Cleveland Clinic, Cleveland, OH

    Background/Purpose: N-Terminal pro-brain natriuretic peptide (NT-proBNP) level tends to correlate with myocardial wall stress and/or myocardial damage (fibrosis). Hence, it has been promoted as a…
  • Abstract Number: 737 • 2017 ACR/ARHP Annual Meeting

    Dynamic Prediction of Pulmonary Hypertension Development in Systemic Sclerosis Patients Using Landmark Analysis – Comparison of Two Models

    Svetlana I. Nihtyanova1, Voon H. Ong2, Emma C. Derrett-Smith3, Benjamin Schreiber4, J. Gerry Coghlan4, Bianca DeStavola5 and Christopher Denton6, 1Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 2Rheumatology, UCL Division of Medicine, London, United Kingdom, 3Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, London, United Kingdom, 4National Pulmonary Hypertension Service, Royal Free Hospital, London, United Kingdom, 5Faculty of Population Health Sciences, UCL Institute of Child Health, London, United Kingdom, 6Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom

    Background/Purpose: Pulmonary hypertension (PH) contributes substantially to systemic sclerosis (SSc)-related morbidity and mortality. It tends to develop later in the disease, creating an opportunity for…
  • Abstract Number: 738 • 2017 ACR/ARHP Annual Meeting

    Prospective Validation of the Systemic Sclerosis Skin Symptoms Patient-Reported Outcome (SSPRO) in a Phase 2 Trial of Anabasum (JBT-101) in Diffuse Cutaneous Systemic Sclerosis (dcSSc)

    Ada Man1, Nancy Dgetluck2 and Barbara White3, 1Rheumatology, University of Manitoba, Winnipeg, MB, Canada, 2Biostatistics, Corbus Pharmaceuticals, Norwood, MA, 3Corbus Pharmaceuticals, Inc., Norwood, MA

    Background/Purpose: Skin thickening is the defining manifestation of dcSSc. A dcSSc patient’s assessment of their skin involvement can provide information about how that patient feels…
  • Abstract Number: 739 • 2017 ACR/ARHP Annual Meeting

    How Effective Is the Home Exercise Program for Hands in Patients with Systemic Sclerosis: Preliminary Results from a Randomized Controlled, Single-Blind, Clinical Trial

    Neslihan Gokcen1, Suade Ozlem Badak2, Tunay Sarpel3, Yasar Sertdemir4 and Eren Erken2, 1Physical Medicine and Rehabilitation, Division of Rheumatology, Cukurova University School of Medicine, Adana, Turkey, 2Internal Medicine, Division of Rheumatology, Cukurova University School of Medicine, Adana, Turkey, 3Physical Medicine and Rehabilitation, Cukurova University School of Medicine, Adana, Turkey, 4Department of Biostatistics and Medical Informatics, Cukurova University School of Medicine, Adana, Turkey

    Background/Purpose: Systemic sclerosis (SSc) represents a heterogeneous autoimmune disease characterized by fibrosis of skin and internal organs. In particular, thickening of the skin, puffy hands,…
  • Abstract Number: 740 • 2017 ACR/ARHP Annual Meeting

    Prediction of Fibrosis Progression in Systemic Sclerosis By Collagen Biomarkers

    Rucsandra Dobrota1, Suzana Jordan2, Pernille Juhl3, Britta Maurer4, Lukas Wildi4, Anne-C. Bay-Jensen5, Morten Karsdal6, Anne Sofie Siebuhr7 and Oliver Distler2, 1Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 3Nordic Bioscience, Herlev, Denmark, 4Department of Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 5Biomarkers and Reseach, Nordic Bioscience, Herlev, Denmark, 6Biomarkers and Reseacrh, Nordic Bioscience, Herlev, Denmark, 7Biomarkers and Research, Nordic Bioscience, Herlev, Denmark, Herlev, Denmark

    Background/Purpose: Altered extracellular matrix (ECM) remodeling leading to fibrosis is a key pathogenic process in systemic sclerosis (SSc). We previously showed that collagen formation and…
  • Abstract Number: 741 • 2017 ACR/ARHP Annual Meeting

    Cardiac MRI in Systemic Sclerosis As Prognostic Tool of Cardiac Mortality in Symptomatic Patients

    Silvia Bosello1, Giovanni Canestrari1, Enrico De Lorenzis1, Gerlando Natalello2, Federico Parisi1, Agostino Meduri3, Riccardo Marano3, Gianfranco Ferraccioli4 and Elisa Gremese5, 1Division of Rheumatology, Università Cattolica - Fondazione Policlinico Universitario A.Gemelli, Rome, Italy, 2Division of Rhematology, Università Cattolica - Fondazione Policlinico Universitario A.Gemelli, Rome, Italy, 3Università Cattolica - Fondazione Policlinico Universitario A.Gemelli, Rome, Italy, 4Institute of Rheumatology, Università Cattolica - Fondazione Policlinico Universitario A.Gemelli, Rome, Italy, 5Division of Rheumatology - Institute of Rheumatology and Affine Sciences, Università Cattolica - Fondazione Policlinico Universitario A.Gemelli, Rome, Italy

    Background/Purpose: Cardiac involvement is a relevant prognostic determinant of outcome in Systemic Sclerosis (SSc). Since the role of cardiac MRI (CMR) is still uncertain, we…
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