Abstracts tagged "scleroderma and systemic sclerosis"

Abstract Number: 903 • 2018 ACR/ARHP Annual Meeting
Riociguat in Patients with Early Diffuse Cutaneous Systemic Sclerosis: A Randomized, Double-Blind, Placebo-Controlled Phase IIb Study (RISE-SSc)
Oliver Distler¹, Yannick Allanore², Christopher P. Denton³, Masataka Kuwana⁴, Marco Matucci-Cerinic⁵, Janet E. Pope⁶, Janethe Pena⁷, Kaisa Laapas⁸, Zhen Yao⁹ and Dinesh Khanna¹⁰, ¹Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ²Rheumatology A Department, Cochin Hospital, Paris Descartes University, Sorbonne Paris Cité, Paris, France, ³UCL Division of Medicine, Royal Free Campus, London, United Kingdom, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶Department of Medicine, University of Western Ontario, London, ON, Canada, ⁷Clinical Development, Bayer US LLC, Whippany, NJ, ⁸StatFinn Oy, Espoo, Finland, ⁹Bayer Healthcare Co. Ltd.,, Beijing, China, ¹⁰Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: There are few disease-modifying therapies for the treatment of systemic sclerosis (SSc), particularly the more severe diffuse cutaneous form (dcSSc). The soluble guanylate cyclase…
Abstract Number: 1108 • 2018 ACR/ARHP Annual Meeting
Antibody Repertoire Dynamics in Systemic Sclerosis after Myeloablative Autologous Hematopoietic Stem-Cell Transplantation or Cyclophosphamide Treatment
Julia Z. Adamska^1,2, Leslie Crofford³, Daniel E. Furst^4,5, Ellen Goldmuntz⁶, Lynette Keyes-Elstein⁷, Maureen D. Mayes⁸, Peter McSweeney⁹, Richard Nash⁹, Ashley Pinckney¹⁰, Beverly Welch¹¹, Keith Sullivan¹² and William H. Robinson^1,2, ¹Stanford University, Stanford, CA, ²VA Palo Alto Health Care System, Palo Alto, CA, ³Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, ⁴University of California Los Angeles, Los Angeles, CA, ⁵University of Washington, Seattle, WA, ⁶NIAID, NIH, Bethesda, MD, ⁷Clinical Statistics, Rho Federal Systems, Inc., Chapel Hill, NC, ⁸Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁹Colorado Blood Cancer Institute, Denver, CO, ¹⁰Rho Federal Systems, Inc., Chapel Hill, NC, ¹¹National Institutes of Health, Bethesda, MD, ¹²Duke University Medical Center, Durham, NC
Background/Purpose: Myeloablative autologous hematopoietic stem-cell transplantation (HSCT) was recently demonstrated to provide benefit over monthly cyclophosphamide (CYC) in the treatment of diffuse cutaneous systemic sclerosis…
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: 1742 • 2018 ACR/ARHP Annual Meeting
Disease-Specific Autoantibodies Associate with Remarkably Different Risk of Development of Significant Lung Fibrosis in Systemic Sclerosis
Svetlana I. Nihtyanova¹, Alper Sari², Anna Leslie³, Voon H. Ong⁴ and Christopher P. Denton⁵, ¹Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, ²Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ³Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, ⁴Division of Medicine, University College London, London, United Kingdom, ⁵University College London, London, United Kingdom
Background/Purpose: Pulmonary fibrosis (PF) is a leading cause of disease-related death in SSc patients. Some studies suggest that the timing of PF development differs between…
Abstract Number: 1878 • 2018 ACR/ARHP Annual Meeting
Immunosuppression in Diffuse Systemic Sclerosis Improves Outcomes Using a Novel Composite Response Index
Boyang Zheng¹, Mianbo Wang² and Murray Baron³, ¹Rheumatology, McGill University Health Center (MUHC), Montreal, QC, Canada, ²Lady Davis Institute for Medical Research, Montreal, QC, Canada, ³Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada
Background/Purpose: Diffuse systemic sclerosis (dcSSc) is a devastating multi-organ disease where the mainstay of treatment is immunosuppression. Data on these therapies are mostly based on…
Abstract Number: 1879 • 2018 ACR/ARHP Annual Meeting
The Effects of Riociguat on Raynaud’s Phenomenon and Digital Ulcers in Patients with Diffuse Systemic Sclerosis: Results from the Phase IIb RISE-SSc Study
Dinesh Khanna¹, Yannick Allanore², Christopher P. Denton³, Masataka Kuwana⁴, Marco Matucci-Cerinic⁵, Janet E. Pope⁶, Janethe Pena⁷, Kaisa Laapas⁸, Zhen Yao⁹ and Oliver Distler¹⁰, ¹Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, ²Rheumatology A Department, Cochin Hospital, Paris Descartes University, Sorbonne Paris Cité, Paris, France, ³UCL Division of Medicine, Royal Free Campus, London, United Kingdom, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ⁵Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁶Department of Medicine, University of Western Ontario, London, ON, Canada, ⁷Clinical Development, Bayer US LLC, Whippany, NJ, ⁸StatFinn Oy, Espoo, Finland, ⁹Bayer AG, Berlin, Germany, ¹⁰Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
Background/Purpose: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of pulmonary arterial hypertension associated with connective tissue disease. It was anticipated that…
Abstract Number: 814 • 2018 ACR/ARHP Annual Meeting
NON Diffuse SSc, Peripheral Neuropathy, Concomitant Sjogren Syndrome and ANTI-RNA Polymerase III Represent Risk Factors for the Higher Frequency of Cancer in a Large Single Cohort of Patients with Systemic Sclerosis
Ana Paula Luppino-Assad¹, Adriana Bortoluzzo², Henrique Carriço da Silva³, Danieli Andrade⁴ and Percival Sampaio-Barros⁴, ¹Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR., sao paulo, Brazil, ²c Instituto Insper de Educação e Pesquisa, São Paulo, SP, Brasil, sao paulo, Brazil, ³Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR., SAO PAULO, Brazil, ⁴Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR., São Paulo, Brazil
Background/Purpose: A higher prevalence of cancer has been described in patients with systemic sclerosis (SSc), but the magnitude of this risk and the type of…
Abstract Number: 775 • 2017 ACR/ARHP Annual Meeting
Transcriptome Sequencing Reveals Genetic Polymorphisms Associated with Ssc Gene Expression Subtypes
Guoshuai Cai¹, Bhaven K. Mehta², Mengqi Huang², Jennifer Franks¹, Tammara A. Wood¹, Kathleen D. Kolstad³, Marianna Stark⁴, Antonia Valenzuela⁵, David Fiorentino⁶, Robert W. Simms⁷, Nicole Orzechowski⁸, Lorinda Chung⁹ and Michael L. Whitfield², ¹Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ²Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ³Rheumatology, Stanford University Medical Center, Stanford, CA, ⁴Stanford University, Stanford, CA, ⁵Immunology and Rheumatology, Stanford University, Palo Alto, CA, ⁶Department of Dermatology, Stanford University School of Medicine, Palo Alto, CA, ⁷Rheumatology, Boston University School of Medicine, Boston, MA, ⁸Dartmouth-Hitchcock Medical Center, Lebanon, NH, ⁹Rheumatology, Stanford University Medical Center, Palo Alto, CA
Background/Purpose: Systemic sclerosis (SSc) is a complex disease characterized by substantial genotypic and phenotypic heterogeneity. Four molecular gene expression subsets have been identified from SSc…
Abstract Number: 919 • 2017 ACR/ARHP Annual Meeting
HLA Type Imputation in the Genome Research in African American Scleroderma Patients (GRASP) Cohort Reveals Strong Associations of African Ancestry MHC Class II Types with Scleroderma and Lack of Class I HLA Type Associations
Elaine F. Remmers¹, Pravitt Gourh², Steven Boyden³, Nadia D. Morgan⁴, Ami A. Shah⁴, Adebowale Adeyemo¹, Amy Bentley¹, Mary A. Carns⁵, Settara C. Chandrasekharappa¹, Lorinda Chung⁶, Lindsey A. Criswell⁷, Chris T. Derk⁸, Robyn T. Domsic⁹, Ayo Doumatey¹, Heather Gladue¹⁰, Avram Goldberg¹¹, Jessica K. Gordon¹², Vivien M Hsu¹³, Reem Jan¹⁴, Dinesh Khanna¹⁵, Maureen D. Mayes¹⁶, Thomas A. Medsger Jr.¹⁷, Paula S. Ramos¹⁸, Marcin A. Trojanowski¹⁹, Lesley A. Saketkoo²⁰, Elena Schiopu¹⁵, Victoria K. Shanmugam²¹, Daniel Shriner¹, Richard M. Silver²², Virginia D. Steen²³, Antonia Valenzuela²⁴, John Varga²⁵, Charles Rotimi¹, Fredrick M. Wigley²⁶, Francesco Boin²⁷ and Daniel L. Kastner²⁸, ¹National Institutes of Health (NIH), National Human Genome Research Institute, Bethesda, MD, ²NIAMS-Rheumatology, National Institutes of Health (NIH), National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Institutes of Health (NIH), National Institute on Deafness and Other Communication Disorders, Bethesda, MD, ⁴Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁵Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ⁶Rheumatology, Stanford University Medical Center, Palo Alto, CA, ⁷Medicine/Rheumatology, University of California, San Francisco, San Francisco, CA, ⁸Rheumatology, University of Pennsylvania, Philadelphia, PA, ⁹Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹⁰Rheumatology, Arthritis and Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹¹NYU Langone Medical Center, New York, NY, ¹²Rheumatology, Hospital for Special Surgery, New York, NY, ¹³University of Medicine and Dentistry of New Jersey--Robert Wood Johnson Medical School, New Brunswick, NJ, ¹⁴Medicine, Rheumatology, University of Chicago, Chicago, IL, ¹⁵University of Michigan, Ann Arbor, MI, ¹⁶University of Texas McGovern Medical School, Houston, TX, ¹⁷Department of Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹⁸Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, ¹⁹Boston University School of Medicine, Boston, MA, ²⁰Rheumatology, Tulane University School of Medicine, New Orleans, LA, ²¹Rheumatology, The George Washington University, Washington, DC, ²²Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, ²³Rheumatology, MedStar Georgetown University Hospital, Washington, DC, ²⁴Stanford University School of Medicine, Stanford, CA, ²⁵Rheumatology and Dermatology, Northwestern University, Feinberg School of Medicine Scleroderma Program, Chicago, IL, ²⁶Rheum Div/Mason F Lord, Johns Hopkins University, Baltimore, MD, ²⁷Rheumatology, University California, San Francisco, San Francisco, CA, ²⁸Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD
Background/Purpose: The Genome Research in African American Scleroderma Patients (GRASP) consortium was created to obtain a collection of African American (AA) scleroderma patients to facilitate…
Abstract Number: 1248 • 2017 ACR/ARHP Annual Meeting
Evaluation of Validated Patient Reported Outcome Measures to Assess Sensitivity to Change in Patients with Systemic Sclerosis and Gastroesophageal Reflux Disease —a Scleroderma Clinical Trials Consortium Collaborative Project
Zsuzsanna McMahan¹, Tracy M. Frech², David Lim³, Veronica J. Berrocal⁴, Cosimo Bruni⁵, Marco Matucci-Cerinic⁶, Vanessa Smith⁷, Karin Melsons⁸, Susanna Proudman⁹, Jinyu Zhang¹⁰, Fabian A Mendoza¹¹, Melanie Woods³ and Dinesh Khanna³, ¹Department of Internal Medicine, Johns Hopkins University, Baltimore, MD, ²Division of Rheumatology, University of Utah, Salt Lake City, UT, ³University of Michigan, Ann Arbor, MI, ⁴Div of Rheumatology, University of Michigan, Ann Arbor, MI, ⁵Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, ⁶Dept of Medicine/Div of Rheum, University of Florence, Firenze, Italy, ⁷Faculty of Internal Medicine, Ghent University, Ghent, Belgium, ⁸Ghent University, Gent, Belgium, ⁹Discipline of Medicine, University of Adelaide, Adelaide, Australia, ¹⁰Thomas Jefferson University, Philadelphia, PA, ¹¹Jefferson Institute of Molecular Medicine and Scleroderma Center, Rheumatology Division, Department of Medicine, Thomas Jefferson University, Philadelphia, PA
Background/Purpose: The UCLA SCTC GIT 2.0 and NIH PROMIS GI Symptoms Scales are validated in scleroderma to assess patient-reported gastrointestinal (GI) involvement. We sought to…
Abstract Number: 1676 • 2017 ACR/ARHP Annual Meeting
Improving Sensitivity to Change of the Modified Rodnan Skin Score over Time
Annel M. Fernandez¹, Robert F. Spiera², Jackie Szymonifka² and Jessica K. Gordon², ¹Medicine- Rheumatology/ Research, Hospital For Special Surgery, New York, NY, ²Rheumatology, Hospital for Special Surgery, New York, NY
Background/Purpose: The Modified Rodnan Skin Score (MRSS) assesses global dermal thickness through the examination of 17 body areas scored by clinical palpation using a 4…
Abstract Number: 1695 • 2017 ACR/ARHP Annual Meeting
Baseline Characteristics and Outcomes in a Retrospective Cohort of Patients with Systemic Sclerosis Related Interstitial Lung Disease
Robert L. Mango¹, Eric L. Matteson², Cynthia S. Crowson³, Jay H. Ryu⁴ and Ashima Makol², ¹Internal Medicine, Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, MN, ²Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, ³Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, ⁴Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN
Background/Purpose: Interstitial lung disease (ILD) is a major contributor to morbidity and mortality in systemic sclerosis (SSc). We sought to identify the distribution of clinical…
Abstract Number: 1713 • 2017 ACR/ARHP Annual Meeting
Defining Genetic Risk for Scleroderma Renal Crisis in RNA-Polymerase III Antibody Positive Patients
Edward Stern¹, Sandra Guerra¹, Harry Chinque¹, David Gonzalez Serna², Markella Ponticos¹, Javier Martin², Maureen D. Mayes³, Shervin Assassi⁴, Carmen Fonseca¹ and Christopher Denton⁵, ¹UCL Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, ²Instituto de Parasitología y Biomedicina López-Neyra, Granada, Spain, ³Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, ⁴University of Texas McGovern Medical School, Houston, TX, ⁵Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom
Background/Purpose: Scleroderma renal crisis (SRC), characterised by accelerated hypertension and acute kidney injury, is a life-threatening complication of systemic sclerosis (SSc). Most SSc cases have…
Abstract Number: 1922 • 2017 ACR/ARHP Annual Meeting
Cell Type Specific Gene Expression Analysis of Early Systemic Sclerosis Skin Shows a Prominent Activation Pattern of Innate and Adaptive Immune System in the Prospective Registry for Early Systemic Sclerosis (PRESS) Cohort
Shervin Assassi¹, Dinesh Khanna², Monique Hinchcliff³, Virginia D. Steen⁴, Faye Hant⁵, Jessica K. Gordon⁶, Ami A. Shah⁷, Jun Ying⁸, William Swindell⁹, Wenjin Zheng¹⁰, Lisha Zhu¹⁰, Victoria K. Shanmugam¹¹, Robyn T. Domsic¹², Flavia V. Castelino¹³, Elana J. Bernstein¹⁴ and Tracy M. Frech¹⁵, ¹University of Texas McGovern Medical School, Houston, TX, ²University of Michigan, Ann Arbor, MI, ³Rheumatology, Northwestern Medicine, Chicago, IL, ⁴Rheumatology, MedStar Georgetown University Hospital, Washington, DC, ⁵Medicine/Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, ⁶Rheumatology, Hospital for Special Surgery, New York, NY, ⁷Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ⁸Department of Internal Medicine - Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁹Dermatology, University of Michigan - Ann Arbor, Ann Arbor, MI, ¹⁰University of Texas - School of Biomedical Informatics, Houston, TX, ¹¹Rheumatology, The George Washington University, Washington, DC, ¹²Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹³Rheumatology, Harvard Medical School, Boston, MA, ¹⁴Rheumatology, Columbia University, New York, NY, ¹⁵Division of Rheumatology, University of Utah, Salt Lake City, UT
Background/Purpose: To examine the global gene expression profile in patients with very early diffuse systemic sclerosis (SSc). Methods: Skin biopsies were obtained from patients enrolled…
Abstract Number: 1923 • 2017 ACR/ARHP Annual Meeting
Single Cell RNA Sequencing Reveals a Signature of Endothelial Injury in Scleroderma Skin
Sokratis Apostolidis¹, Giuseppina Stifano², Tracy Tabib³, Lisa Rice², Christina Morse³, Bashar Kahaleh⁴ and Robert A. Lafyatis³, ¹Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, ²Boston University School of Medicine, Boston, MA, ³Medicine, Rheumatology, University of Pittsburgh Medical Center, Pittsburgh, PA, ⁴Medicine/Rheumatology, University of Toledo, Toledo, OH
Background/Purpose: Vascular injury is a hallmark event in the pathogenesis of Systemic Sclerosis (SSc). Endothelial dysfunction happens early in the course of the disease and…

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