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  • Abstract Number: 1716 • 2018 ACR/ARHP Annual Meeting

    Evaluation of American College of Rheumatology Provisional Composite Response Index in Systemic Sclerosis in a Phase II Trial of Abatacept Vs. Placebo

    Dinesh Khanna1 and Cathie Spino2, 1Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, 2Biostatistics, University of Michigan, Ann Arbor, MI

    Background/Purpose: Treatment with CTLA4Ig, abatacept (ABA), in early diffuse cutaneous systemic sclerosis (dcSSc; the Phase 2 ASSETtrial) showed evidence of improvements in modified Rodnan skin…
  • Abstract Number: 1717 • 2018 ACR/ARHP Annual Meeting

    Expert Consensus on the Screening, Treatment, and Management of Patients with Systemic Sclerosis-Interstitial Lung Disease, and the Potential Role of Anti-Fibrotics in a Treatment Paradigm for Systemic Sclerosis-Interstitial Lung Disease: A Delphi Consensus Study

    Dinesh Khanna1,2, Mary Strek3, Brian Southern4, Rajan Saggar5, Vivien Hsu6, Maureen D. Mayes7, Rick Silver8, Virginia D. Steen9, Donald Zoz10 and Franck Rahaghi11, 1Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, 2Division of Rheumatology, University of Michigan, Ann Arbor, MI, 3Pulmonology, University of Chicago, Chicago, IL, 4Pulmonology, Cleveland Clinic Ohio, Cleavland, OH, 5Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 6Rheumatology, Robert Wood Johnson University Scleroderma Program, New Brunswick, NJ, 7Rheumatology, University of Texas Medical School at Houston, Houston, TX, 8Rheumatology, Medical University of SC, Charleston, SC, 9Dept of Rheumatology, Georgetown Univ Medical Center, Washington, DC, 10Pulmonology, Boehringer Ingelheim, Ridgefield, CT, 11Cleveland Clinic Florida Weston, Weston, FL

    Background/Purpose: Systemic sclerosis (SSc), or scleroderma, is a rare, autoimmune, multisystem connective tissue disorder characterized by vascular damage, autoimmunity, and fibrosis of the skin and…
  • Abstract Number: 1718 • 2018 ACR/ARHP Annual Meeting

    Skin Gene Expression Profiling Predicts Longitudinal Modified Rodnan Skin Score Change

    Chase Correia1, Mary A. Carns2, Kathleen Aren2, Monique Hinchcliff3 and J. Matthew Mahoney4, 1Rheumatology, Northwestern Feinberg School of Medicine, Chicago, IL, 2Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 3Rheumatology, Northwestern University, Chicago, IL, 4Neurological Sciences, University of Vermont College of Medicine, Burlington, VT

    Background/Purpose: Systemic sclerosis (SSc) causes varying degrees of skin fibrosis with varying trajectory. Extent of skin involvement is measured by the modified Rodnan skin score…
  • Abstract Number: 1719 • 2018 ACR/ARHP Annual Meeting

    High-Throughput Quantitative Histology in Systemic Sclerosis Skin Disease Using Computer Vision

    Chase Correia1, Seamus Mawe2, Mary A. Carns3, Kathleen Aren3, Aileen Hoffman4, Monique Hinchcliff5 and J. Matthew Mahoney6, 1Northwestern University, Chicago, IL, 2Department of Neurological Sciences, University of Vermont Larner College of Medicine, Burlington, VT, 3Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, 4Northwestern Feinberg School of Medicine, Chicago, IL, 5Rheumatology, Northwestern University, Chicago, IL, 6Neurological Sciences, University of Vermont College of Medicine, Burlington, VT

    Background/Purpose: The lack of a reproducible and quantitative method to accurately assess histological skin fibrosis in patients with systemic sclerosis (SSc) has long plagued clinical…
  • Abstract Number: 1720 • 2018 ACR/ARHP Annual Meeting

    Higher Neutrophil Count Predicts More Severe Skin/Lung Disease and Increased Mortality in Early Systemic Sclerosis

    Rana Taherian1, Vishnu Mohan1, Jun Ying2, Samuel Theodore1, Julio Charles1, Hau Pham1, Minghua Wu3, Maureen D. Mayes3 and Shervin Assassi3, 1University of Texas Health Science Center at Houston, Houston, TX, 2Department of Internal Medicine - Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 3Rheumatology, University of Texas Health Science Center at Houston, Houston, TX

    Background/Purpose: Our recent global blood transcript studies show that systemic sclerosis (SSc) patients have a prominent neutrophil signature.  Mechanistic studies with neutrophils are hampered by…
  • Abstract Number: 1721 • 2018 ACR/ARHP Annual Meeting

    Racial Differences in SSc Disease Presentation: A Cross-Sectional European Scleroderma Trials and Research Group Study

    Veronika K. Jaeger1, Elise Siegert2, Eric Hachulla3, Paolo Airò4, Gabriele Valentini5, Marco Matucci-Cerinic6, Oliver Distler7, Franco Cozzi8, Yannick Allanore9, Mangtao Li10, Mohammed Tikly11 and Ulrich A. Walker1, 1Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 2Rheumatology and Clinical Immunology, Charité - University Medicine Berlin, Berlin, Germany, 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, 5Second University of Naples, Naples, Italy, 6Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, 7Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 8Division of Rheumatology, Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy, 9Immunogenetics, Cochin Institute, Paris, France, 10Peking Union Medical College Hospital, Beijing, China, 11Division of Rheumatology, Department of Internal Medicine, Chris Hani Baragwanath Hospital and University of the Witwatersrand, Johannesburg, South Africa

    Background/Purpose: Genetic and environmental factors play a significant role in SSc. African Americans are known for a higher SSc incidence, an earlier age of onset,…
  • Abstract Number: 1722 • 2018 ACR/ARHP Annual Meeting

    Patient-Level Factors Associated with Hospital Readmission Among Patients with Systemic Sclerosis Associated Pulmonary Hypertension

    Kimberly Showalter1, Laura Pinheiro2, Jackie Szymonifka3, Irina Sobol4, Virginia D. Steen5 and Jessica K. Gordon1, 1Rheumatology, Hospital for Special Surgery, New York, NY, 2Internal Medicine, Weill Cornell Medicine, New York, NY, 3Hospital for Special Surgery, New York, NY, 4Cardiology, Weill Cornell Medicine, New York, NY, 5Rheumatology, MedStar Georgetown University Hospital, Washington, DC

    Background/Purpose: Repeat hospitalizations in patients with pulmonary hypertension from all causes are associated with worse survival. However, predictors of systemic sclerosis associated pulmonary hypertension (SSc-PH)…
  • Abstract Number: 1723 • 2018 ACR/ARHP Annual Meeting

    Intensified B-Cell Depletion Therapy in Progressive Systemic Sclerosis Patients: 24 Months Follow-up

    Daniela Rossi1, Irene Cecchi2, Massimo Radin3, Elena Rubini4, Savino Sciascia5 and Dario Roccatello6, 1Department of Medicine and Experimental Oncology, CMID - Center of Research of Immunopathology and Rare Diseases, Turin, Italy, 2Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Turin, Italy, 3Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Turin, Italy, 4Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bosco Hospital, Turin, Italy., Turin, Italy, 5Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Italy, Torino, Italy, 6Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin and S. Giovanni Bo, Turin, Italy

    Background/Purpose:Systemic sclerosis (SSc) is a connective tissue autoimmune disease with systemic involvement and a serious medical condition with a high rate of mortality, especially due…
  • Abstract Number: 1724 • 2018 ACR/ARHP Annual Meeting

    Effectiveness and Safety of Rituximab for the Treatment of Refractory Systemic Sclerosis Associated Calcinosis: A Case Series

    Francisco Javier Narváez1, Juan Pablo Pirola2, Judit Lluch3, Pablo Juárez3, Isabel Morales3 and Joan Miquel Nolla3, 1Rheumatology Department, Hospital de Bellvitge. Barcelona. Spain, L’Hospitalet de Llobregat, Spain, 2Rheumatology, Hospital Privado Centro Médico de Córdoba, Cordoba, Argentina, 3Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain

    Background/Purpose: To analyze the effectiveness and safety of rituximab (RTX) for the treatment of refractory systemic sclerosis (SSc)–associated calcinosis. Methods: We undertook an observational study…
  • Abstract Number: 1725 • 2018 ACR/ARHP Annual Meeting

    Fecal Microbiota Transplantation in Patients with Systemic Sclerosis- a Pilot Study

    Håvard Fretheim1, Oyvind Midtvedt1, Anders Heiervang Tennøe1, Henriette Didriksen1, Torhild Garen1, Espen Bækkevold1, Johannes R. Hov1, Knut EA Lundin2, Marius Trøseid1, Øyvind Molberg1 and Anna-Maria Hoffmann-Vold1, 1Oslo University Hospital, Oslo, Norway, 2Dept of gastroenterology, Oslo University Hospital, Oslo, Norway

    Background/Purpose: Up to 90% of patients with systemic sclerosis (SSc) have symptoms from the gastrointestinal (GI) tract. Earlier studies have shown a distinct alteration of…
  • Abstract Number: 1726 • 2018 ACR/ARHP Annual Meeting

    Sexual Dysfunction in Systemic Sclerosis Female Patients

    Barbora Hermankova1,2, Maja Spiritovic1,2, Hana Smucrova2, Sabina Oreska2,3, Hana Storkanova2,3, Karel Pavelka3,4, Jiri Vencovsky2,5, Ladislav Šenolt2,3, Radim Becvar2,3 and Michal Tomcik2,3, 1Department of Physiotherapy, Faculty of Physical Education and Sports, Charles University, Prague, Czech Republic, Prague, Czech Republic, 2Institute of Rheumatology, Prague, Czech Republic, Prague, Czech Republic, 3Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, 4Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, 5Department Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disease leading to various physical and psychological impairments including sexual dysfunction. The aim of this study was…
  • Abstract Number: 1727 • 2018 ACR/ARHP Annual Meeting

    Prospective Confirmation of Potential Link between Proton Pump Inhibitor Exposure and Calcinosis in Systemic Sclerosis

    Lauren V. Host1, Corrado Campochiaro1, Svetlana I. Nihtyanova2, Christopher P. Denton3 and Voon H. Ong1, 1Rheumatology and Connective Tissue Diseases, University College London, Royal Free Hospital, London, United Kingdom, 2Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, 3University College London, London, United Kingdom

    Background/Purpose: Long-term use of proton pump inhibitors (PPI) have been linked to safety concerns. In a previous retrospective analysis, a potential association between PPI use…
  • Abstract Number: 1728 • 2018 ACR/ARHP Annual Meeting

    Pharmacokinetics of Cyclophosphamide in Scleroderma Treated By Cyclophosphamide Versus Transplantation

    David Adams1, Keith Sullivan2, Ivan Spasojevic1, Ping Fan1, Mario Sampson3, Michael Cohen-Wolkowiez3, E. William St Clair4, Rob Woolson5, Jan Storek6, Mary Ellen Csuka7, Ashley Pinckney5, Beverly Welch8, Ellen Goldmuntz9, Daniel E. Furst10, Leslie Crofford11, Lynette Keyes-Elstein12, Maureen Mayes13, Peter McSweeney14 and Richard Nash14, 1Medicine, Duke University Medical Center, Durham, NC, 2Duke University Medical Center, Durham, NC, 3Pediatrics-Infectious Disease, Duke University Medical Center, Durham, NC, 4Rheumatology, Duke University Medical Center, Durham, NC, 5Rho Federal Systems, Inc., Chapel Hill, NC, 6University of Calgary, Calgary, AB, Canada, 7Medicine, Medical College of Wisconsin, Milwaukee, WI, 8National Institutes of Health, Bethesda, MD, 9NIAID, NIH, Bethesda, MD, 10University of Washington, Seattle, WA, 11Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, 12Clinical Statistics, Rho Federal Systems, Inc., Chapel Hill, NC, 13Rheumatology, University of Texas at Houston, Houston, TX, 14Colorado Blood Cancer Institute, Denver, CO

    Background/Purpose: The pharmacokinetics (PK) of cyclophosphamide (CP) and its primary active metabolite, 4-hydroxycyclophosphamide (4-OH-CP) have not been adequately studied in scleroderma. The hypothesis of this…
  • Abstract Number: 1729 • 2018 ACR/ARHP Annual Meeting

    Increased Risk of Valvular Heart Disease in Patients with Systemic Sclerosis: Results from a Population-Based Cohort (1980-2016)

    Reto Kurmann1, Avneek Singh Sandhu2, Cynthia S. Crowson3, Rekha Mankad1, Thomas Osborn2, Kenneth J. Warrington4 and Ashima Makol2, 1Cardiovascular Diseases, Mayo Clinic College of Medicine and Science, Rochester, MN, 2Rheumatology, Mayo Clinic, Rochester, MN, 3Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, 4Rheumatology, Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, MN

    Background/Purpose: Systemic Sclerosis (SSc) is a complex, heterogenous autoimmune disease characterized by microvascular injury and widespread fibrosis of the skin and internal organs, particularly lungs…
  • Abstract Number: 1730 • 2018 ACR/ARHP Annual Meeting

    A Role for Duplex Ultrasound of Hand Arteries in the Assessment of the Vasculopathy Associated to Systemic Sclerosis-like Diseases

    Silvia Pérez Esteban1, Esperanza Naredo Sánchez1, Sheila Recuero Díaz1, Fredeswinda I. Romero-Bueno2, Gabriel Herrero-Beaumont3 and Olga Sanchez-Pernaute4, 1Rheumatology, Fundación Jiménez Díaz University Hospital & Health Research Institute, Madrid, Spain, 2Section for Autoimmune Diseases, Rheumatology, Fundación Jiménez Díaz University Hospital & Health Research Institute, Madrid, Spain, 3Bone and Joint Research Unit, Fundación Jiménez Díaz University Hospital & Health Research Institute, Madrid, Spain, 4Rheumatology Division. Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain

    Background/Purpose: Small vessel arteriopathy precedes the development of organ damage and tissue fibrosis in systemic sclerosis (SSc) and related disorders. It is currently considered that…
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