Abstracts tagged "scleroderma and systemic sclerosis"

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…
Abstract Number: 2665 • 2017 ACR/ARHP Annual Meeting
Mortality in an Early Diffuse Cutaneous Systemic Sclerosis Cohort—Data from the Prospective Registry for Early Systemic Sclerosis
Tracy M. Frech¹, Shervin Assassi², Elana J. Bernstein³, Flavia V. Castelino⁴, Robyn T. Domsic⁵, Jessica K. Gordon⁶, Faye Hant⁷, Monique Hinchcliff⁸, Bernie LaSalle⁹, Victoria K. Shanmugam¹⁰, Virginia D. Steen¹¹ and Dinesh Khanna¹², ¹Division of Rheumatology, University of Utah, Salt Lake City, UT, ²University of Texas McGovern Medical School, Houston, TX, ³Department of Medicine, Division of Rheumatology, Columbia University, New York, NY, ⁴Rheumatology, Allergy, Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, ⁵Medicine - Rheumatology, University of Pittsburgh, Pittsburgh, PA, ⁶Rheumatology, Hospital for Special Surgery, New York, NY, ⁷Medicine/Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC, ⁸Department of Medicine Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, ⁹University of Utah, Salt Lake City, UT, ¹⁰Rheumatology, The George Washington University, Washington, DC, ¹¹Division of Rheumatology, Department of Medicine, MedStar Georgetown University Hospital, Washington, DC, ¹²University of Michigan, Ann Arbor, MI
Background/Purpose: The Prospective Registry of Early Systemic Sclerosis (PRESS) cohort is an early diffuse cutaneous systemic sclerosis (dcSSc) that provides an opportunity to assess the…
Abstract Number: 2674 • 2017 ACR/ARHP Annual Meeting
The Association between the Extent of Skin Thickness and Organ Involvement, Function and Quality of Life in Early Diffuse Cutaneous Systemic Sclerosis
Janet E. Pope¹, Murray Baron², Tatiana Nevskaya³, Carl Baxter⁴ and Dena Ramey⁵, ¹Medicine, Divsion of Rheumatology, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada, ²Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada, ³Rheumatology Division, St. Joseph’s Health Care London, London, ON, Canada, ⁴Global Outcomes Research, MSD Ltd, Hoddesdon, United Kingdom, ⁵Epidemiology, Merck & Co., Upper Gwynedd, PA
Background/Purpose: To estimate whether severity of skin thickness is associated with disease severity, function, quality of life (QoL) and progression of internal organ involvement over…
Abstract Number: 257 • 2017 ACR/ARHP Annual Meeting
The Use of Positron Emission Tomography (PET)-Scan for the Quantitative Assessment of Interstitial Lung Disease in Systemic Sclerosis
Daphne Peelen¹, Ben Zwezerijnen², Esther Nossent¹, Lilian Meijboom¹, Otto Hoekstra³, Conny van der Laken⁴ and Alexandre Voskuyl⁴, ¹VUmc, Amsterdam, Netherlands, ²Nuclear Medicine, VUmc, Amsterdam, Netherlands, ³Department of Radiology & Nuclear Medicine, VU University Medical Center, Amsterdam, The Netherlands, Amsterdam, Netherlands, ⁴Department of Rheumatology, Amsterdam Rheumatology and immunology Center - location VU University Medical Center, Amsterdam, The Netherlands, Amsterdam, Netherlands
Background/Purpose: Interstitial lung disease (ILD) in systemic sclerosis is treated by immunosuppressive drugs (e.g. cyclophosphamide), aimed at reduction of inflammatory response . Differentiation between inflamed…
Abstract Number: 2690 • 2017 ACR/ARHP Annual Meeting
Plasma D-Dimer Levels Are More Frequently Elevated in Limited Than Diffuse Cutaneous Systemic Sclerosis but Do Not Reflect Disease Duration or Vasculopathy
Anna Gill¹, Svetlana I. Nihtyanova², Pratima Chowdary³ and Christopher Denton¹, ¹Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, ²Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, ³Department of Haematology, Royal Free Hospital, London, United Kingdom
Background/Purpose: D-dimers are degradation products of cross linked fibrin and are biomarkers of activation of the coagulation system. Plasma D-dimer levels can be raised in…
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: 2691 • 2017 ACR/ARHP Annual Meeting
Increased Circulating Cadherin-11 Levels in Patients with Systemic Sclerosis
Bochra Jandali¹, Grace H. Lo², Robert L. Welschhans³, Julio Charles⁴, Ramona Mihu^3,5, Maureen D. Mayes⁶, Shervin Assassi⁷ and Sandeep K. Agarwal³, ¹Medicine, Section of Immmunology, Allergy and Rheumatology, Baylor College of Medicine, Houston, TX, ²Immunology, Allergy, Rheumatology, Baylor College of Medicine, Houston, TX, ³Medicine, Section of Immunology, Allergy and Rheumatology, Baylor College of Medicine, Houston, TX, ⁴Internal Medicine/Rheumatology, University of Texas - Houston Medical School, Houston, TX, ⁵Medicine, Division of Rheumatology, Houston Methodist Hospital, Houston, TX, ⁶Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, ⁷University of Texas McGovern Medical School, Houston, TX
Background/Purpose: Systemic sclerosis (SSc) is a chronic systemic disease characterized by skin and internal organs fibrosis along with vasculopathy. Previous studies have reported increased cadherin-11…
Abstract Number: 6L • 2016 ACR/ARHP Annual Meeting
Myeloablative Autologous Transplantation of CD34+ -Selected Hematopoietic Stem Cells (HSCT) Vs Monthly Intravenous Cyclophosphamide (CYC) for Severe Scleroderma with Internal Organ Involvement: Outcomes of a Randomized North American Clinical Trial
Keith Sullivan¹, Lynette Keyes-Elstein², Peter McSweeney³, Ashley Pinckney², Beverly Welch⁴, Maureen D Mayes⁵, Richard Nash³, Leslie J. Crofford⁶, Sharon Castina², Linda Griffith⁷, Ellen Goldmuntz⁸ and Daniel E. Furst⁹, ¹Duke University, Durham, NC, ²Rho, Inc, Chapel Hill, NC, ³Colorado Blood Cancer Institute, Denver, CO, ⁴6610 Rockledge Dr., NIAID/NIH, Bethesda, MD, ⁵Department of Internal Medicine - Rheumatology, University of Texas-McGovern Medical School, Houston, TX, ⁶Medicine, Vanderbilt University Medical Center, Nasville, TN, ⁷NIAID, NIH, Bethesda, MD, ⁸NIAID, National Institutes of Health, Bethesda, MD, ⁹David Geffen School of Medicine at UCLA, Los Angeles, CA
Background/Purpose: Therapeutic options for diffuse cutaneous systemic sclerosis (dcSSc) are limited. The Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial was a multicenter study designed to…
Abstract Number: 836 • 2016 ACR/ARHP Annual Meeting
Comprehensive Characterization of Calcinosis in a Multicenter International Cohort of Patients with Systemic Sclerosis
Antonia Valenzuela^1,2, Jessica K. Gordon³, Tatiana Sofia Rodriguez-Reyna⁴, Susanna Proudman^5,6, 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…

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