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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 1861 • ACR Convergence 2021

    Epidermal Growth Factor Receptor Pathway and Fibrosis in Systemic Sclerosis Skin

    Miruna Carnaru1, Monique Hinchcliff2, Ian Odell1 and F. Perry Wilson3, 1Yale School Of Medicine, New Haven, CT, 2Yale School of Medicine, Westport, CT, 3Section of Nephrology, Department of Medicine, Yale School of Medicine, New Haven, CT; Department of Medicine Clinical and Translational Research Accelerator Yale School of Medicine, New Haven, CT

    Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disorder with chronic morbidity and high mortality. Disease pathogenesis involves microvascular damage, immune dysregulation, and fibrosis affecting…
  • Abstract Number: 0404 • ACR Convergence 2021

    Perifollicular Hypopigmentation in Systemic Sclerosis: Associations with Clinical Features and Internal Organ Involvement

    Melody Chung1, Jamie Perin1, Carrie Richardson2, Christopher Mecoli3, Fred Wigley1 and Zsuzsanna McMahan4, 1Johns Hopkins University, Baltimore, MD, 2Rush University Medical Center, Chicago, IL, 3Johns Hopkins University School of Medicine, Baltimore, MD, 4Johns Hopkins Rheumatology, Baltimore, MD

    Background/Purpose: Among the autoimmune rheumatic diseases, there is precedent for utilizing cutaneous manifestations of systemic disease as clues to clinical phenotype and disease prognosis. Though…
  • Abstract Number: 0551 • ACR Convergence 2021

    Metabolic Intermediate Dimethyl-Alpha-Ketoglutarate Is a Novel Repressor of Pathogenic Myofibroblast Reprogramming and Skin Fibrosis in Systemic Sclerosis

    Blaž Burja1, Ishani Banik2, Shervin Assassi3, Michael Whitfield4, J. Matthew Mahoney5, Andreja Erman6, Matija Tomšič7, Mitchell P. Levesque2, Snežna Sodin-Šemrl7, Gabriela Kania8, Hubert Rehrauer9, Ziga Rotar7, Oliver Distler10, Katja Lakota11 and Mojca Frank-Bertoncelj10, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich and Department of Rheumatology, University Medical Centre Ljubljana, Zurich, Switzerland, 2Department of Dermatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 3University of Texas McGovern Medical School at Houston, Houston, TX, 4Geisel School of Medicine, Lebanon, NH, 5Center for Quantitative Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 6Institute for Cell Biology, University Medical Centre Ljubljana, Ljubljana, Slovenia, 7Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, 8Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 9Functional Genomics Center Zurich, ETH Zurich and University of Zurich, Zurich, Switzerland, 10Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, 11Department of Rheumatology, University Medical Centre Ljubljana, Llubljana, Slovenia

    Background/Purpose: Metabolic perturbations drive fibroblast-to-myofibroblast reprogramming and tissue fibrosis. Restoring perturbed metabolism might represent a new antifibrotic strategy. Here we explored the capacity of dimethyl-alpha-ketoglutarate…
  • Abstract Number: 1048 • ACR Convergence 2021

    Estimating the Weight of Rheumatologic Diseases in Mortality in Spain: Basic Cause of Death vs Multiple Cause Analysis

    ana Perez1, Fernando Albarran2, Cristina Bohorquez2, Atusa Movasat2, Lucia Ruiz2, Paula Pretel2, elena Rabadan2, Valentina Emperiale2, Adrian abbasi3, julio suarez3, lorena montano3, emilio rico3, alfredo prieto4, Inmaculada Leon5, melchor Alvarez de Mon3 and beatriz perez6, 1Hospital Universitario Príncipe de Asturias. Rheumatology. Medicine Faculty. Alcala University Department., Madrid, Spain, 2Hospital Universitario Príncipe de Asturias, Rheumatology Department, Alcala de Henares, 3Hospital Universitario Príncipe de Asturias, Madrid, Spain, 4Faculty of Medicine. Alcala University, Alcala de Henares, Spain, 5Communicable Diseases. Instituto de Salud Carlos III, Madrid, Spain, 6Department of Epidemiology of Chronic Diseases.National Center of Epidemiology/CIBERESP. Instituto de Salud Carlos III, Madrid, Spain

    Background/Purpose: Classical statistics provide information on mortality rates for basic causes of death. Although many inflammatory rheumatic diseases decrease life expectancy, they are generally not…
  • Abstract Number: 1384 • ACR Convergence 2021

    A Genomic Meta-Analysis of Clinical Variables and Association with Intrinsic Molecular Subsets in Systemic Sclerosis

    Jennifer Franks1, Diana Toledo1, Viktor Martyanov1, Yue Wang2, Suiyuan Huang3, Tammara Wood1, Cathie Spino3, Robyn Domsic4, Monique Hinchcliff5, Dinesh Khanna3 and Michael Whitfield6, 1Geisel School of Medicine at Dartmouth, Hanover, NH, 2Geisel School of Medicine at Dartmouth, West Lebanon, NH, 3University of Michigan, Ann Arbor, MI, 4University of Pittsburgh, Pittsburgh, PA, 5Yale School of Medicine, Westport, CT, 6Geisel School of Medicine, Lebanon, NH

    Background/Purpose: Four intrinsic molecular subsets (Inflammatory, Fibroproliferative, Limited, Normal-like) have been identified in systemic sclerosis (SSc) that may have different clinical associations. To test this…
  • Abstract Number: 1841 • ACR Convergence 2021

    Presence of Autoantibodies to Dense-Fine-Speckled 70 (DFS70) Do Not Necessarily Rule out Connective Tissue Diseases

    Louisa-Marie Mockenhaupt1, Ramona Dolscheid-Pommerich1, Charlotte Behning1, Birgit Stoffel-Wagner2, Peter Brossart1 and Valentin Schäfer1, 1University Hospital Bonn, Bonn, Germany, 2University Hospital Bonn, Bonn

    Background/Purpose: Antinuclear antibodies (ANA) are serological markers for the presence of connective tissue diseases [1]. In some patients, a pattern can be detected in ANA…
  • Abstract Number: 0405 • ACR Convergence 2021

    Retrospective Study on the Prognostic Value of Cardiac Magnetic Resonance Imaging Abnormalities in Systemic Sclerosis

    Hao Cheng Shen1, Catherine Faucher2, Anne Chin3, Carl Chartrand-Lefebvre3, Ragui Chehata4, Julia Cadrin-Tourigny5, Francois-Pierre Mongeon6, Jean-Paul Makhzoum7, Océane Landon-Cardinal8, Josiane Bourré-Tessier8, Éric Rich9, Jean-Richard Goulet8, Paul R Fortin10, Jean-Luc Senécal8 and Sabrina Hoa8, 1Department of Medicine, Université de Montréal, Montréal, QC, Canada, 2Department of Medicine, Université Laval, Québec, QC, Canada, 3Department of Radiology, Centre hospitalier de l’Université de Montréal, Montréal, QC, Canada, 4Department of Medicine, Université de Montréal, Montreal, Montréal, QC, Canada, 5Division of Non-Invasive Cardiology, Department of Specialized Medicine, Montreal Heart Institute; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 6Division of electrophysiology, Department of Specialized Medicine, Montreal Heart Institute; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 7Division of Internal Medicine, Hôpital Sacré-Coeur de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 8Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, 9Division of Rheumatology, Centre hospitalier de l’Université de Québec-Université Laval, Montréal, QC, Canada, 10CHU de Quebec - Universite Laval, Québec City, QC, Canada

    Background/Purpose: Cardiac involvement is a leading cause of death in systemic sclerosis (SSc). Cardiac MRI (CMR) is useful in the early assessment of cardiac disease.…
  • Abstract Number: 0554 • ACR Convergence 2021

    Reverse Signaling Through PD-L1 Plays a Central Role in Extracellular Matrix Protein Secretion from Cutaneous Myofibroblasts in Systemic Sclerosis

    Maithri Aspari1, Stinne Greisen1, Malene Hvid1, Klaus Sondergaard2, Voon Ong3, Christopher Denton4, David Abraham5 and Bent Deleuran1, 1Aarhus University, Aarhus, Denmark, 2Aarhus University Hospital, Aarhus, Denmark, 3University College London Medical School Royal Free Campus, London, United Kingdom, 4University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, 5University College London, London, United Kingdom

    Background/Purpose: The PD-1/PD-L1 pathway has been implicated in Systemic Sclerosis (dSSc). This disease is dominated by increased extracellular matrix deposition initiated by myofibroblasts. We therefore…
  • Abstract Number: 1099 • ACR Convergence 2021

    Characteristics and Outcomes of Myocardial Infarction in Patients with Rheumatoid Arthritis, Systemic Lupus Erythematosus, Systemic Sclerosis, Gout and Osteoarthritis Patients Using the National Inpatient Sample Database from 2002-2018

    Sonia Gupta1, Vinay thallapally2, Sarah Aurit2, Rouhin sen3 and Joseph Nahas2, 1Creighton University School of Medicine, Omaha, NE, 2Creighton University, Omaha, NE, 3Colorado University Anschutz Medical Campus, Denver, CO

    Background/Purpose: Patients with the autoimmune rheumatic disease have an increased risk of acute myocardial infarction (MI). Our study was designed to compare the prevalence, characteristics…
  • Abstract Number: 1385 • ACR Convergence 2021

    Overall Survival in Patients with Systemic Autoimmune Diseases Following Lung or Heart-Lung Transplantation at a Single High-Volume Academic Transplant Center: A Comparative Cohort Study

    Jason Melehani1, Shufeng Li2, Joshua Mooney3 and Lorinda Chung2, 1Stanford University, San Jose, CA, 2Stanford University, Palo Alto, CA, 3Stanford University, Stanford, CA

    Background/Purpose: Lung manifestations of systemic autoimmune diseases are a frequent cause of early death. For many patients, current treatments cannot arrest the inexorable progression to…
  • Abstract Number: 1842 • ACR Convergence 2021

    Peripheral Blood Cell Gene Expression Profiling Predicts Response to Mycophenolate in Systemic Sclerosis Related Interstitial Lung Disease

    Shervin Assassi1, Wenjin Zheng2, Elizabeth Volkmann3, Xuan Wang4, Holly Wilhalme5, Marka Lyons6, Michael Roth5 and Donald Tashkin5, 1University of Texas McGovern Medical School at Houston, Houston, TX, 2School of Biomedical Informatics at the University of Texas Health Science Center at Houston, Houston, TX, 3Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, 4Baylor Scott & White Health, Dallas, TX, 5University of California Los Angeles, Los Angeles, CA, 6Division of Rheumatology, University of Texas McGovern Medical School at Houston, Houston, TX

    Background/Purpose: Response to immunosuppression is variable in systemic sclerosis (SSc) related interstitial lung disease (ILD). Contrary to pulmonary tissue, peripheral blood cell (PBC) RNA can…
  • Abstract Number: 0142 • ACR Convergence 2021

    Description of a Scleroderma Cohort and Management of Lung Disease Risk at a Rural Academic Medical Center

    Jenna Marinock1, P. Daniel Nicholas2, Kirsten Koons3, Rakesh Kriplani4, Andrea Berger4 and David Bulbin5, 1Geisinger Commonwealth School of Medicine, Philadelphia, PA, 2Geisinger Medical Center, Danville, PA, 3Geisinger Medical Center, Lewisburg, PA, 4Department of Population Health Sciences, Geisinger Medical Center, Danville, PA, 5Geisinger Health System, Danville, PA

    Background/Purpose: Systemic sclerosis interstitial lung disease (SSc-ILD) and pulmonary hypertension (SSc-PH) are leading causes of morbidity and mortality in patients with SSc. Few screening guidelines…
  • Abstract Number: 0406 • ACR Convergence 2021

    Gender Differences in Clinical Features and Outcomes of Systemic Sclerosis: Analysis of Reuma.pt/SSc Registry

    Raquel Freitas1, Patrícia Martins2, Eduardo Dourado2, Tânia Santiago3, Francisca Guimarães4, Bruno Fernandes5, Salomé Garcia6, Beatriz Samões7, Ana Pinto8, Nuno Gonçalves9, Maria Lourenço10, Emanuel Costa11, Margarida Rocha12, Maura Couto13, Ana Catarina Duarte1, Filipe Araújo14, Inês Cordeiro2, Fátima Godinho1, Catarina Resende15, Maria Joo Salvador16, Ana Cristina Cordeiro1 and Maria José Santos17, 1Hospital Garcia de Orta, Rheumatology department, Almada, Portugal, 2Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, Lisbon Academic Medical Centre and European Reference Network on Rare Connective Tissue and Musculoskeletal Diseases Network (ERN-ReCONNET); Rheumatology Research Unit, Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal, 3Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, 4Unidade Local de Saúde do Alto Minho, Rheumatology department, Ponte de Lima, Portugal, 5Rheumatology Department, Centro Hospitalar Universitário de São João EPE, Braga, Portugal, 6Rheumatology Department, Centro Hospitalar Universitário de São João EPE, Porto, Portugal, 7Rheumatology Department, Centro Hospitalar Vila Nova de Gaia, VIla Nova de Gaia, Portugal, 8Local Health Unit of Guarda, Portugal, Barcelos, Portugal, 9Centro Hospitalar Lisboa Ocidental, Rheumatology Department; Hospital Central do Funchal, Lisboa, Portugal, 10Hospital Egas Moniz, Barcelos, Portugal, 11Hospital de Braga, Braga, Portugal, 12Centro Hospitalar Universitário Lisboa Norte, Rheumatology Department, Faro, Portugal, 13Centro Hospitalar Tondela - Viseu, Rheumatology Department, Linda a Velha, Portugal, 14Hospital de Sant'Ana, Rheumatology and Osteoporosis department, Parede, Portugal, 15Centro Hospitalar Universitário Lisboa Norte, Rheumatology Department, Lisbon, Portugal, 16Centro Hospitalar e Universitário de Coimbra, Rheumatology Department, Coimbra, Portugal, 17Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal

    Background/Purpose: Systemic sclerosis (SSc) is a rare connective tissue disease of unknown etiology, with a broad spectrum of clinical and laboratory features. Evidence for the…
  • Abstract Number: 0555 • ACR Convergence 2021

    Pathway-Driven Drug Repositioning in Systemic Sclerosis from Omics Data

    Dillon Popovich1, Tamar Abel1, Noelle Kosarek2, Monica Espinoza3, Rezvan Parvizi4, Jonathan Garlick5 and Michael Whitfield4, 1Geisel School of Medicine, West Lebanon, NH, 2Dartmouth Geisel School of Medicine, Hartford, VT, 3Geisel School of Medicine, Hanover, NH, 4Geisel School of Medicine, Lebanon, NH, 5Tufts University School of Dental Medicine, Boston, MA

    Background/Purpose: Omic analyses of systemic sclerosis (SSc) skin biopsy datasets identified distinct sets of patients that respond differently to treatment. The goal of this study…
  • Abstract Number: 1136 • ACR Convergence 2021

    Risk of Osteoporosis in Patients with Systemic Sclerosis: A Cross-Sectional Analysis of Two European Prospective Cohorts

    Charles Midol1, Edgar Wiebe2, Elise Siegert2, Dörte Huscher3, David Launay4, Eric Hachulla4, Vincent Sobanski1 and Frank Buttgereit5, 1Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000 Lille, France, Lille, France, 2Department of Rheumatology and Clinical Immunology, Charité – Universitätsmedizin Berlin, Germany, Berlin, Germany, 3Institute of Biometry and Clinical Epidemiology, and Berlin Institute of Health, Charité – Universitätsmedizin Berlin, Berlin, Germany, 4Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000 Lille, France, Lille Cedex, France, 5Charité – Universitätsmedizin Berlin, Berlin, Germany

    Background/Purpose: Patients with systemic sclerosis (SSc) are at high risk for osteoporosis (OPO) and fragility fractures, although their exact prevalences are unknown. This might be…
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