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  • Abstract Number: 829 • 2016 ACR/ARHP Annual Meeting

    High Level of Chemokine CCL2 Is Associated with Lung Fibrosis Progression and Reduced Survival in Two Independent Systemic Sclerosis Cohorts

    Anna Hoffmann-Vold1, Richard Huyen2, Elizabeth R. Volkmann2, Oyvind Midtvedt1, Vyacheslav Palchevskiy2, May Brit Lund3, Torhild Garen1, Trond Mogens Aalokken4, Anders Heiervang Tennøe1, Stephen Samuel Weigt2, Mike Shino2, Rajan Saggar5, David Ross2, Joseph Lynch III2, Thor Ueland6, Michael Fishbein7, Pål Aukrust8, Øyvind Molberg1 and John A Belperio2, 1Rheumatology, Oslo University Hospital, Oslo, Norway, 2University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 3Respiratory Medicine, Oslo University Hospital, Oslo, Norway, 4Radiology, Oslo University Hospital, Oslo, Norway, 5Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 6Research, Oslo University Hospital, Oslo, Norway, 7Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA, 8Research Intitute for Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway

    Background/Purpose:  Markers for early identification of progressive interstitial lung disease (ILD) in systemic sclerosis (SSc) are in demand. The proto-typical inflammatory chemokine CCL2 has been linked…
  • Abstract Number: 830 • 2016 ACR/ARHP Annual Meeting

    Cardiopulmonary Disease Development in Anti-RNA Polymerase III Positive Systemic Sclerosis; Comparative Analyses from an Unselected, Prospective Patient Cohort

    Anna Hoffmann-Vold1, Anders Heiervang Tennøe2, Oyvind Midtvedt2, May Brit Lund3, Torhild Garen2, Fadi El-Hage4, Trond Mogens Aalokken5, Eli Taraldsrud4 and Øyvind Molberg2, 1Division of Rheumatology, Oslo University Hospital, Oslo, Norway, 2Rheumatology, Oslo University Hospital, Oslo, Norway, 3Respiratory Medicine, Oslo University Hospital, Oslo, Norway, 4Oslo University Hospital, Oslo, Norway, 5Radiology, Oslo University Hospital, Oslo, Norway

    Background/Purpose: Extensive skin disease and renal crisis are hallmarks of anti-RNA polymerase III (RNAP) positive systemic sclerosis (SSc), while data on lung and heart involvement…
  • Abstract Number: 831 • 2016 ACR/ARHP Annual Meeting

    Immunosuppression May Prevent Interstitial Lung Disease in Systemic Sclerosis

    Sabrina Hoa1, Marie Hudson2, Mianbo Wang3, Russell Steele4, Murray Baron5 and Canadian Scleroderma Research Group, 1Division of Rheumatology, Jewish General Hospital, Lady Davis Institute, Montreal, QC, Canada, 2Jewish General Hospital, Lady Davis Institute and McGill University, Montreal, QC, Canada, 3Lady Davis Institute for Medical Research, Montreal, QC, Canada, 4Mathematics, McGill University, Montreal, QC, Canada, 5Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada

    Background/Purpose: Interstitial lung disease (ILD) is a leading cause of premature mortality in systemic sclerosis (SSc). Immunosuppression is used for treatment of established disease. However,…
  • Abstract Number: 832 • 2016 ACR/ARHP Annual Meeting

    Defining Skin Ulcers in Systemic Sclerosis: A Systematic Literature Review of Skin Ulcer Definitions and a Preliminary Consensus-Based New SSc Skin Ulcer Definition

    Yossra A Suliman1, Cosimo Bruni2, Sindhu R. Johnson3, Emanuela Praino4, Mohamed Alemam5, Nabeel Borazan6, Laura Cometi7, Bethany Myers8, Dinesh Khanna9, Yannick Allanore10, Murray Baron11, Thomas Krieg12, Ariane L. Herrick13, Suzanne Kafaja14, Christopher Denton15, Marco Matucci Cerinic16 and Daniel E. Furst17, 1Rheumatology and Rehabilitation dept., Rheumatology and Rehabilitation dept. Assiut university hospital, Assiut Egypt, Assiut, Egypt, 2Department of Biomedicine, Division of Rheumatology AOUC, Excellence Centre for Research, Florence, Italy, 3Rheumatology, Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, 4DIM, Rheumatology Unit, Bari, Italy, 5Clinical Pathology and Laboratory Medicine Department, Assistant Lecturer, Qena, Egypt, 6Medicine, David Geffen School of Medicine, Los Angeles, CA, 7Division of Rheumatology, Department of Experimental and Clinical Medicine,, Florencei, Italy, 8UCLA Louise M. Darling Biomedical Library, Research Informationist, Los Angeles, CA, 9University of Michigan, Ann Arbor, MI, 10Immunogenetics, Cochin Institute, Paris, France, 11Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada, 12Universität zu Köln, Köln, Germany, 13Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Manchester, United Kingdom, 14Medicine/Rheumatology, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 15Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 16Department of BioMedicine, Division of Rheumatology, Transition Unit, University of Florence, Firenze, Italy, 17University of California, Los Angeles, Los Angeles, CA

    Background/Purpose: Skin ulcers in SSc are a major clinical challenge and there are various un-validated definitions of skin ulcers utilized in SSc-related clinical trials. We…
  • Abstract Number: 833 • 2016 ACR/ARHP Annual Meeting

    A Small Proportion of Patients with Systemic Sclerosis with Suspected Pulmonary Hypertension Meet the Published Inclusion/Exclusion Criteria for Two Systemic Sclerosis Associated Pulmonary Arterial Hypertension Screening Algorithms– Results from a Single Center Cohort

    Amber Young1, Victor Moles2, Vivek Nagaraja3, Scott H. Visovatti4, Vallerie McLaughlin4 and Dinesh Khanna5, 1Department of Internal Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, MI, 2Department of Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, 3Department of Medicine [Division of Rheumatology], University of Toledo, Toledo, OH, 4Internal Medicine, Division of Cardiology, University of Michigan, Ann Arbor, MI, 5University of Michigan, Ann Arbor, MI

    Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). Active screening detects SSc-PAH earlier and…
  • Abstract Number: 834 • 2016 ACR/ARHP Annual Meeting

    Distinctive Clinical Phenotype of Anti-Centromere Antibody Positive Diffuse Systemic Sclerosis

    Joana Caetano1, Svetlana Nihtyanova2, Jennifer Harvey3, Christopher P.Denton4 and Voon H. Ong5, 1Department of Medicine IV, Systemic Immunomediated Diseases Unit, Fernando Fonseca Hospital, Amadora, Portugal, 2Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Hospital, London, United Kingdom, 3Clinical Immunology, Royal Free Hospital, London, United Kingdom, 4Centre of Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 5Rheumatology, UCL Division of Medicine, London, United Kingdom

    Background/Purpose: Although anti-centromere antibodies (ACA) typically associate with limited cutaneous subset of systemic sclerosis (lcSSc) this reactivity is also seen in some cases with diffuse…
  • Abstract Number: 835 • 2016 ACR/ARHP Annual Meeting

    A Normal Pulmonary Diffusion Capacity Is Rare in Pulmonary Artery Hypertension in Systemic Sclerosis

    Rebecca Overbury1, Tracy M. Frech2, Maureen Murtaugh3, Virginia D. Steen4 and PHAROS investigators, 1Internal Medicine and Pediatrics, University of Utah, Salt Lake City, UT, 2Division of Rheumatology, University of Utah, Salt Lake City, UT, 3University of Utah, Salt Lake, UT, 4Rheumatology, Georgetown University Medical Center, Washington, DC

    Background/Purpose:   Methods:   Results:  > 70% (mean 78%) versus 191 patients (94.6%) who had a low DLCO <70% (mean 39%). There was no difference…
  • Abstract Number: 836 • 2016 ACR/ARHP Annual Meeting

    Comprehensive Characterization of Calcinosis in a Multicenter International Cohort of Patients with Systemic Sclerosis

    Antonia Valenzuela1,2, Jessica K. Gordon3, Tatiana Sofia Rodriguez-Reyna4, Susanna Proudman5,6, Murray Baron7, Monique Hinchcliff8, Dinesh Khanna9, Amber Young10, Flavia V. Castelino11, Sara R. Schoenfeld12, Virginia D. Steen13, David Fiorentino14 and Lorinda Chung15, 1Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA, 2Stanford University School of Medicine, Stanford, CA, 3Rheumatology, Hospital for Special Surgery, New York, NY, 4Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, 5Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia, 6Discipline of Medicine, University of Adelaide, Adelaide, Australia, 7Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada, 8Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 9University of Michigan, Ann Arbor, MI, 10Department of Internal Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, MI, 11Rheumatology, Allergy, Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 12Rheumatology Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, 13Rheumatology, Georgetown University Medical Center, Washington, DC, 14Department of Dermatology, Stanford University School of Medicine, Palo Alto, CA, 15Rheumatology, Stanford University Medical Center, Palo Alto, CA

    Background/Purpose: Calcinosis cutis is a debilitating complication of systemic sclerosis (SSc) affecting one quarter of patients. Repetitive trauma may be involved in the pathogenesis of…
  • Abstract Number: 837 • 2016 ACR/ARHP Annual Meeting

    Development of a Skin-Specific Scleroderma Patient Reported Outcome Instrument

    Ada Man1, Jeannette K. Correa2, Jessica Ziemek3, David T. Felson4 and Robert Lafyatis5, 1Rheumatology, University of Manitoba, Winnipeg, MB, Canada, 2Psychology, Boston University, Boston, MA, 3Rheumatology, Boston University, Boston, MA, 4Clinical Epidemiology Unit, Boston University School of Medicine, Boston, MA, 5Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA

    Background/Purpose: Skin fibrosis is the hallmark of SSc and may lead to significantly reduced quality of life in ways that may not be directly proportional…
  • Abstract Number: 838 • 2016 ACR/ARHP Annual Meeting

    Esophageal Dysmotility and Interstitial Lung Disease in Patients with Scleroderma: A Retrospective Study

    Shweta Kishore1, Santhanam Lakshminarayanan1, Chia-Ling Kuo2 and Ranadeep Mandhadi1, 1Division of Rheumatology, University of Connecticut, Farmington, CT, 2Department of Community Medicine and Health Care, University of Connecticut, Farmington, CT

    Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease with pulmonary involvement seen in 75% of patients and esophageal involvement in 90% of the patients.…
  • Abstract Number: 839 • 2016 ACR/ARHP Annual Meeting

    Immunosuppression Does Not Prevent Severe Gastrointestinal Disease in Systemic Sclerosis

    Nicolas Richard1, Marie Hudson2, Mianbo Wang3, Murray Baron4, Genevieve Gyger1 and Canadian Scleroderma Research Group, 1McGill University, Jewish General Hospital, Montreal, QC, Canada, 2Medicine/Rheumatology, Jewish General Hospital, Lady Davis Research Institute, Montreal, QC, Canada, 3Lady Davis Institute for Medical Research, Montreal, QC, Canada, 4Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada

    Background/Purpose: Severe gastrointestinal (GI) disease is associated with considerable morbidity and high mortality in systemic sclerosis (SSc). There are no known preventative treatments. We wished…
  • Abstract Number: 840 • 2016 ACR/ARHP Annual Meeting

    Time Trends in Incidence and Mortality of Systemic Sclerosis in Denmark from 1995-2011: A Nationwide Cohort Study

    Sheraz Butt1, Charlotte Andersson2, Søren Jacobsen3, Gunnar Gislason4 and Christian Torp-Pedersen5, 1Department of Medicine, Glostrup Hospital, Glostrup, Denmark, 2Department of Medicine, Glostrup Hospital, Copenhagen, Denmark, 3Center for Rheumatology and Spine Diseases, Rigshospitalet - Glostrup, University of Copenhagen, Denmark, Glostrup, Denmark, 4Department of Cardiology, Gentofte Hospital, Copenhagen, Denmark, 5Aalborg University, Aalborg, Denmark

    Time Trends In Incidence And Mortality Of Systemic Sclerosis In Denmark From 1995-2011: A Nationwide Cohort Study Background/Purpose: Epidemiological studies have documented an increasing incidence…
  • Abstract Number: 841 • 2016 ACR/ARHP Annual Meeting

    Low Nucleoside Triphosphate Pyrophosphohydrolase Activity Contributes to Pathologic Mineralization in Systemic Sclerosis

    Yue Ding1, Supraja Yeturi1, Claudia Gohr1, Mary Ellen Csuka1 and Ann K. Rosenthal2,3, 1Medicine, Medical College of Wisconsin, Milwaukee, WI, 2Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI, 3Medicine, Clement J. Zablocki VA Medical Center, Milwaukee, WI

    Background/Purpose: Calcinosis is a major source of morbidity in patients with systemic sclerosis (SS). In addition, increased coronary calcification has been reported in patients with…
  • Abstract Number: 842 • 2016 ACR/ARHP Annual Meeting

    Aminaphtone Treatment Increases Skin Blood Perfusion and Related Clinical Symptoms in Patients Affected By Raynaud’s Phenomenon: A Pilot Study Based on Laser Speckle Contrast Analysis

    Barbara Ruaro, Sabrina Paolino, Carmen Pizzorni, Maurizio Cutolo and Alberto Sulli, Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, Genoa, Italy, Genova, Italy

    Background/Purpose: Aminaphtone is an oral vasoactive drug used to treat microvascular impairement since 30 years, and recently suggested to down-regulate endothelin-1 production by endothelial cells,…
  • Abstract Number: 843 • 2016 ACR/ARHP Annual Meeting

    Forced Vital Capacity Predicts Outcome in Scleroderma Associated Interstitial Lung Disease with Concomitant Pulmonary Hypertension:  Data from the Pharos Registry

    Joyce Sujin Lee1, Jessica K. Gordon2, Jackie Szymonifka3, Virginia Steen4 and Aryeh Fischer5, 1SOM-MED, University of Colorado, Denver - Anschutz Medical Campus, Aurora, CO, 2Rheumatology, Hospital for Special Surgery, New York, NY, 3Epidemiology and Biostatistics, Hospital for Special Surgery, New York, NY, 4Georgetown University School of Medicine, Washington, DC, 5Medicine / Center for Lungs and Breathing, University of Colorado School of Medicine, Aurora, CO

    Forced vital capacity predicts outcome in scleroderma associated interstitial lung disease with concomitant pulmonary hypertension:  Data from the PHAROS registry Background/Purpose:   Interstitial lung disease…
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