Abstracts tagged "Scleroderma"

Abstract Number: 1646 • 2019 ACR/ARP Annual Meeting
Incidence Rate and Prevalence of Valvular Heart Disease in Systemic Sclerosis: A Retrospective Cohort Study from a Single Institution
Mamoun Elnagar¹, Yasser Radwan ¹, Mohamed Elwazir ², Reto Kurmann ², Rekha Mankad ¹, Thomas Osborn ¹, Ashima Makol ¹, Leroy Griffing ³, Cynthia Crowson ² and Alicia Hinze ¹, ¹Mayo Clinic Minnesota, Rochester, MN, ²Mayo Clinic Minnesota, Rochester, ³Mayo Clinic Arizona, Scottsdale
Background/Purpose: Systemic sclerosis (SSc) associated heart involvement remains ill-defined, with non-ischemic systolic heart failure, conduction defects, and arrhythmias often included in the definition in clinical…
Abstract Number: 2750 • 2019 ACR/ARP Annual Meeting
Development and Preliminary Validation of a Novel Lung Ultrasound Interpretation Criteria for the Detection of Interstitial Lung Disease in Patients with Systemic Sclerosis
Robert Fairchild¹, Diana Yang ², Melody Chung ¹, Laurel Sharpless ¹, Shufeng Li ³ and Lorinda Chung ¹, ¹Stanford University, Palo Alto, CA, ²Santa Clara Valley Medical Center, Palo Alto, CA, ³Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), and screening, characterization, and monitoring of disease activity are important for therapeutic…
Abstract Number: 1049 • 2019 ACR/ARP Annual Meeting
Induction of a Profibrotic Phenotype in Normal Dermal Fibroblasts by Expression of PIM1 Kinase and Demonstration of Antifibrotic Effects of Inhibition of PIM Kinases in Systemic Sclerosis Dermal Fibroblasts
Danielle Pomante ¹, Sergio Jimenez ² and Sonsoles Piera-Velazquez¹, ¹Thomas Jefferson University, Philadelphia, PA, ²Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Thomas Jefferson University, Philadelphia, PA, Philadelphia, PA
Background/Purpose: There is an urgent unmet need for effective therapeutic approaches for Systemic Sclerosis (SSc), a systemic autoimmune disease characterized by progressive fibrosis of skin…
Abstract Number: 1647 • 2019 ACR/ARP Annual Meeting
The Collaborative National Quality and Efficacy Registry for Scleroderma: Data Completion Outcomes from a Multicenter United States Cohort Using Guideline-Based Registry Practices
Victoria Shanmugam¹, Tracy Frech ², Virginia Steen ³, Laura Hummers ⁴, Ami Shah ⁵, Elana Bernstein ⁶, Dinesh Khanna ⁷, Jessica Gordon ⁸, Flavia Castelino ⁹, Lorinda Chung ¹⁰, Faye Hant ¹¹, Emily Startup ¹², John VanBuren ¹², Luke Evnin ¹³ and Shervin Assassi ¹⁴, ¹George Washington University, Georgetown, DC, ²Division of Rheumatology, University of Utah and Salt Lake VAMC, Salt Lake City, UT, ³Georgetown University, Washington, D.C., USA, Georgetown, ⁴Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, ⁵Johns Hopkins Hospital, Baltimore, MD, ⁶Division of Rheumatology, Columbia University, New York, NY, ⁷Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ⁸Hospital for Special Surgery, New York, NY, ⁹Harvard University, Boston, MA, ¹⁰Stanford University, Palo Alto, CA, ¹¹Medical University of South Carolina, Charleston, SC, ¹²University of Utah, Salt Lake City, UT, ¹³Scleroderma Research Foundation, San Francisco, CA, ¹⁴Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX
Background/Purpose: The Collaborative National Quality and Efficacy Registry (CONQUER) for Scleroderma is a multicenter US-based longitudinal study of patients with systemic sclerosis (SSc) within 5…
Abstract Number: 2916 • 2019 ACR/ARP Annual Meeting
Ancestry-Specific Classical HLA Alleles Define Phenotypic Subsets in the African American Scleroderma Population
Pravitt Gourh ¹, Sarah Safran², Steven Boyden ³, Ami Shah ⁴, Maureen Mayes ⁵, Ayo Doumatey ⁶, Amy Bentley ⁶, Daniel Shriner ⁶, Robyn Domsic ⁷, Thomas Medsger Jr ⁸, Paula Ramos ⁹, Richard Silver ¹⁰, Virginia Steen ¹¹, John Varga ¹², Vivien Hsu ¹³, Lesley Saketkoo ¹⁴, Elena Schiopu ¹⁵, Dinesh Khanna ¹⁶, Jessica Gordon ¹⁷, Brynn Kron ¹⁸, Lindsey Criswell ¹⁹, Heather Gladue ²⁰, Chris Derk ²¹, Elana Bernstein ²², S Louis Bridges ²³, Victoria Shanmugam ²⁴, Kathleen Kolstad ²⁵, Lorinda Chung ²⁶, Suzanne Kafaja ²⁷, Reem Jan ²⁸, Marcin Trojanowski ²⁹, Avram Goldberg ³⁰, Benjamin Korman ³¹, Peter Steinbach ³², Settara Chandrasekharappa ⁶, James Mullikin ⁶, Adebowale Adeyemo ⁶, Charles Rotimi ⁶, Frederick Wigley ³³, Daniel Kastner ³⁴, Francesco Boin ³⁵, Elaine Remmers ⁶ and Theresa Alexander ³⁶, ¹National Institutes of Rheumatology, Bethesda, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), Bethesda, MD, ³University of Utah, SALT LAKE CITY, UT, ⁴Johns Hopkins Hospital, Baltimore, MD, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁶National Human Genome Research Institute (NHGRI), Bethesda, MD, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸University of Pittsburg School of Medicine, Pittsburg, PA, ⁹Medical University of South Carolina, Charleston, ¹⁰Division of Rheumatology, Medical University of South Carolina, Charleston, SC, ¹¹Georgetown University, Washington, D.C., USA, Georgetown, ¹²Northwestern University, Chicago,, IL, ¹³Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ, ¹⁴New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans; Tulane University School of Medicine, University Medical Center – Comprehensive Pulmonary Hypertension Center, USA, New Orleans, ¹⁵Department of Rheumatology, University of Michigan, Ann Arbor, ¹⁶Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ¹⁷Hospital for Special Surgery, New York, NY, ¹⁸University of California San Francisco, San Francisco, CA, ¹⁹University of California, San Francisco, San Francisco, ²⁰Arthritis & Osteoporosis Consultants Of The Carolinas, Charlotte, NC, ²¹University of Pennsylvania, Philadelphia, PA, ²²Division of Rheumatology, Columbia University, New York, NY, ²³University of Alabama at Birmingham, Birmingham, ²⁴George Washington University, Georgetown, DC, ²⁵Division of Immunology and Rheumatology, Department of Medicine, Stanford University, Stanford, CA, ²⁶Stanford University, Palo Alto, CA, ²⁷Department of Medicine. Rheumatology Division. UCLA, Los Angeles, CA, ²⁸University of Chicago Pritzker School of Medicine, Chicago, IL, ²⁹Boston University Medical Center, Boston, MA, ³⁰New York University Langone Medical Center, New York, NY, ³¹University of Rochester Medical Center, Rochester, NY, ³²Center for Molecular Modeling, Center for Information Technology, National Institutes of Health, Bethesda, MD, ³³Johns Hopkins University, Division of Rheumatology, Baltimore, ³⁴National Institutes of Health, Bethesda, MD, ³⁵UCSF, San Francisco, CA, ³⁶University of Maryland, College Park, MD
Background/Purpose: Systemic sclerosis (SSc), or scleroderma, is a heterogeneous disease that is divided into limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) forms based on the…
Abstract Number: 1053 • 2019 ACR/ARP Annual Meeting
Parallel Analysis of Systemic Sclerosis and Keloidal Morphea Skin Biopsies Delineates the Hallmark Profibrotic Gene Expression Profile for Scleroderma in Vivo
Kristina Clark¹, Corrado Campochiaro ¹, Nataliya Gak ¹, Emma Derrett-Smith ¹ and Christopher Denton ², ¹University College London, London, United Kingdom, ²University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: We have examined whole skin biopsy gene expression by RNAseq in a rare subgroup of scleroderma with both systemic sclerosis (SSc) and concurrent keloidal…
Abstract Number: 1659 • 2019 ACR/ARP Annual Meeting
Linaclotide for the Treatment of Gastrointestinal Symptoms in Systemic Sclerosis
Eric Dein¹, John Clarke ², Frederick Wigley ³ and Zsuzsanna McMahan* ³, ¹Johns Hopkins, Baltimore, MD, ²Stanford, Pao Alto, ³Johns Hopkins University, Division of Rheumatology, Baltimore
Background/Purpose: Gastrointestinal (GI) involvement is the most common internal organ affected in systemic sclerosis (SSc). Constipation is a common GI complication in SSc that affects…
Abstract Number: 2917 • 2019 ACR/ARP Annual Meeting
Geographic Disparities in Systemic Sclerosis Mortality in the United States: 1999 to 2017
Alicia Rodriguez-Pla¹ and Robert W Simms ², ¹University of Arizona/Banner Health Medical Center, Tucson, AZ, ²Boston University, Boston, MA
Background/Purpose: Population mortality studies in the United States have previously reported a progressive increase in the scleroderma (SSc) mortality rates from 1959 to 2002. Identification…
Abstract Number: 710 • 2019 ACR/ARP Annual Meeting
Relationship Between High-Resolution Computer Tomography and FVC% Predicted for Classification of Pulmonary Hypertension in Systemic Sclerosis
Sara Jaafar¹, Paul Cronin ², Dharshan Vummidi ¹, Scott Visovatti ¹, Amber Young ³, Suiyuan Huang ¹, Vallerie McLaughlin ⁴ and Dinesh Khanna ⁵, ¹University of Michigan, Ann Arbor, MI, ²Unviersity of Michigan, Ann Arbor, MI, ³University of Michigan, Ann Arbot, ⁴Univeristy of Michigan, Ann Arbor, MI, ⁵Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor
Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in scleroderma-spectrum disorders (SSc). FVC% has been used to differentiate Group 1…
Abstract Number: 1054 • 2019 ACR/ARP Annual Meeting
Analysis of Serum Markers Across the Scleroderma Spectrum Shows Subset and Stage Specific Profiles of Fibrogenesis
Kristina Clark¹, Corrado Campochiaro ¹, Katherine Nevin ², Eszter Csomor ², Nicholas Galwey ², Mary Morse ², Nicolas Wisniacki ², Shaun Flint ², Voon Ong ³, Emma Derrett-Smith ¹ and Christopher Denton ⁴, ¹University College London, London, United Kingdom, ²GlaxoSmithKline, Stevenage, United Kingdom, ³UCL Medical School, Royal Free Campus, London, ⁴University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: Systemic sclerosis (SSc) is characterised by autoimmunity, fibrosis and vasculopathy. There is striking heterogeneity in skin fibrosis that is likely to reflect the balance…
Abstract Number: 1831 • 2019 ACR/ARP Annual Meeting
Subtypes of Scleroderma Lung Involvement Associated with Burden of Disease and Outcomes
Sarah French¹, Kim Taylor ¹, Stephanie Rush ² and Francesco Boin ¹, ¹UCSF, San Francisco, CA, ²University of California, San Francisco, San Francisco, CA
Background/Purpose: Pulmonary disease is a leading cause of mortality in scleroderma (SSc). Previous studies indicate that the coexistence of pulmonary hypertension (PH) and interstitial lung…
Abstract Number: 124 • 2018 ACR/ARHP Annual Meeting
M10, a Small Fragment of the Hepatocyte Growth Factor Receptor, Attenuates Fibrotic Changes in a Murine Model of Scleroderma Lung Disease and in Human Lung Fibroblasts
Tanjina Akter¹, Ilia Atanelishvili¹, Atsushi Noguchi², Galina S. Bogatkevich¹ and Rick Silver³, ¹Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, ²Division of Rheumatology, Endocrinology and Nephrology,, Hokkaido University Graduate School of Medicine, Sapporo, Japan, ³Rheumatology, Medical University of SC, Charleston, SC
Background/Purpose: Interstitial lung disease (ILD) is the major cause of mortality among scleroderma (systemic sclerosis, SSc) patients. Extracellular matrix (ECM) deposition is a hallmark of…
Abstract Number: 130 • 2018 ACR/ARHP Annual Meeting
The Role of Cofilin, an Actin Associated Protein, in Activation of Systemic Sclerosis Vascular Smooth Muscle Cells
Shadia Nada, Bashar Kahaleh and Nezam Altorok, Medicine/Rheumatology, University of Toledo, Toledo, OH
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by activation of the immune system, vascular dysfunction and tissue fibrosis. Vascular dysfunction in…
Abstract Number: 811 • 2018 ACR/ARHP Annual Meeting
Identification of Risk Factors for Gastric Antral Vascular Ectasia (GAVE) Among Systemic Sclerosis Patients
Naomi Serling-Boyd¹, Shufeng Li², David Fiorentino², Laren Becker³, Nielsen Fernandez-Becker³, John Clarke⁴ and Lorinda Chung^5,6, ¹Medicine, Stanford University School of Medicine, Stanford, CA, ²Dermatology, Stanford University School of Medicine, Stanford, CA, ³Gastroenterology, Stanford University School of Medicine, Palo Alto, CA, ⁴Gastroenterology, Stanford University School of Medicine, Stanford, CA, ⁵VA Palo Alto Health Care System, Palo Alto, CA, ⁶Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto, CA
Background/Purpose: Gastric Antral Vascular Ectasia (GAVE) is a vascular manifestation of systemic sclerosis (SSc) characterized by erythematous streaks and longitudinal rugal folds in the stomach…
Abstract Number: 902 • 2018 ACR/ARHP Annual Meeting
Longitudinal Trends in Clinical Disease Features after Myeloablative Autologous Stem-Cell Transplantation or Cyclophosphamide in Severe Scleroderma
Lynette Keyes-Elstein¹, Ellen Goldmuntz², Ashley Pinckney³, Leslie Crofford⁴, Daniel E. Furst⁵, Maureen D. Mayes⁶, Peter McSweeney⁷, Richard Nash⁷, Beverly Welch⁸ and Keith Sullivan⁹, ¹Biostatistics, Rho Federal Systems, Inc, Chapel Hill, NC, ²NIAID, NIH, Bethesda, MD, ³Rho Federal Systems, Inc., Chapel Hill, NC, ⁴Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, TN, ⁵UCLA, Los Angeles, CA, ⁶Rheumatology, University of Texas McGovern Medical School, Houston, TX, ⁷Colorado Blood Cancer Institute, Denver, CO, ⁸6610 Rockledge Dr., NIAID/NIH, Bethesda, MD, ⁹Duke University Medical Center, Durham, NC
Background/Purpose: The Scleroderma: Cyclophosphamide or Transplantation (SCOT) study established the long-term superiority of hematopoietic stem cell transplant (HSCT) over cyclophosphamide (CYC) [Sullivan KM, et al.…

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