Abstracts tagged "Scleroderma"

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: 711 • 2019 ACR/ARP Annual Meeting
Ultrasound Evaluation of the Hands in Patients with Systemic Sclerosis: Osteophytosis Is a Major Contributor to Tender Joints
Robert Fairchild¹, Melody Chung ¹, Laurel Sharpless ¹, Shufeng Li ², Jison Hong ¹, Khushboo Sheth ¹ and Lorinda Chung ¹, ¹Stanford University, Palo Alto, CA, ²Division of Immunology and Rheumatology, Department of Medicine, Stanford, Palo Alto, CA
Background/Purpose: Systemic Sclerosis (SSc) is a progressive fibrotic and vascular disease with peripheral manifestations including arthritis, tendinopathy, sclerodactyly, contractures, calcinosis, acroosteolysis, and vascular disease which…
Abstract Number: 1057 • 2019 ACR/ARP Annual Meeting
Dissecting the Cellular Mechanism of Prostacyclin Analog Iloprost in Reversing Vascular Dysfunction in Scleroderma
Pei-Suen Tsou¹, Nicholas Flavahan ² and Dinesh Khanna ³, ¹University of Michigan, Ann Arbor, MI, ²The Johns Hopkins University School of Medicine, Baltimore, MD, ³Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence, University of Michigan, Ann Arbor
Background/Purpose: Iloprost improves Raynaud‘s phenomenon and digital ulcers in scleroderma (SSc) patients. This is hypothesized to reflect anti-platelet and vasodilatory effects. Different trials and cohorts…
Abstract Number: 1833 • 2019 ACR/ARP Annual Meeting
Efficacy and Safety of Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease by Use of Mycophenolate at Baseline: Subgroup Analysis of the SENSCIS Trial
Kristin Highland¹, Oliver Distler ², Masataka Kuwana ³, Yannick Allanore ⁴, Shervin Assassi ⁵, Arata Azuma ⁶, Arnaud Bourdin ⁷, Christopher Denton ⁸, Jörg Distler ⁹, Anna Maria Hoffmann-Vold ¹⁰, Dinesh Khanna ¹¹, Maureen Mayes ⁵, Ganesh Raghu ¹², Madelon Vonk ¹³, Martina Gahlemann ¹⁴, Mannaig Girard ¹⁵, Susanne Stowasser ¹⁶, Donald Zoz ¹⁷, Aryeh Fischer ¹⁸ and Toby Maher ¹⁹, ¹Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ²Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ³Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, ⁴Dept. of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁶Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ⁷PhyMedExp, University of Montpellier, INSERM U1046, CNRS UMR 9214 and Department of Respiratory Diseases, University of Montpellier, CHU Montpellier, Montpellier, France, Montpellier, ⁸University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom, ⁹Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ¹⁰Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ¹¹Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ¹²University of Washington, Seattle, USA, Seattle, ¹³Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands, Nijmegen, Gelderland, Netherlands, ¹⁴Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ¹⁵Boehringer Ingelheim France S.A.S., Reims, France, Reims, France, ¹⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ¹⁷Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, Connecticut, USA, Ridgefield, CT, ¹⁸University of Colorado School of Medicine, Denver, Colorado, USA, Denver, CO, ¹⁹National Heart and Lung Institute, Imperial College London, UK and National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, London, United Kingdom
Background/Purpose: In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib reduced the annual rate of decline in forced vital capacity…
Abstract Number: 712 • 2019 ACR/ARP Annual Meeting
Safety and Tolerability of Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease in the SENSCIS Trial: Subgroup Analysis Based on Demographic Characteristics
Oliver Distler¹, Kristin Highland ², Maureen Mayes ³, Masataka Kuwana ⁴, Lesley Saketkoo ⁵, Madelon Vonk ⁶, Michael Kreuter ⁷, Laura Hummers ⁸, Ute von Wangenheim ⁹, Martina Gahlemann ¹⁰, Veronika Kohlbrenner ¹¹, Margarida Alves ¹², Emmanuelle Clerisme-Beaty ¹² and Arata Azuma ¹³, ¹Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland, ²Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH, ³Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, Bunkyo-ku, Tokyo, Japan, ⁵New 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, ⁶Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands, Nijmegen, Gelderland, Netherlands, ⁷Center for Interstitial and Rare Lung Diseases, Pneumology, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Germany, Germany, Germany, ⁸Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, USA, Baltimore, MD, ⁹Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany, Biberach an der Riss, ¹⁰Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland, Basel, Switzerland, ¹¹Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA, Ridgefield, ¹²Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim am Rhein, Germany, ¹³Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, Tokyo, Japan
Background/Purpose: In the SENSCIS trial in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD), the adverse events associated with nintedanib were manageable for most patients…
Abstract Number: 1059 • 2019 ACR/ARP Annual Meeting
BDCA2 Targeting of Human Plasmacytoid Dendritic Cells via CBS004 Reverts Dependent IFN Activation and Tissue Fibrosis in vitro and in vivo
Rebecca Ross¹, Clarissa Corinaldesi ², Gemma Migneco ³, Yasser El-Sherbiny ⁴, Steve Holmes ⁵, Jörg Distler ⁶, Clive McKimmie ⁷ and Francesco Del Galdo ⁸, ¹Univesristy of Leeds, Leeds, England, United Kingdom, ²University of Leeds, Leeds, England, United Kingdom, ³University of Leeds, Leeds, United Arab Emirates, ⁴University of Nottingham, Nottingham, United Kingdom, ⁵Capella Bioscience, london, United Kingdom, ⁶Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁷University of Leeds, Leeds, United Kingdom, ⁸University of Leeds and LTHT NIHR Biomedical Research Centre, Leeds, United Kingdom
Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…
Abstract Number: 1834 • 2019 ACR/ARP Annual Meeting
Serum Interferon Chemokine Score Predicts Better Response to Immunosuppression in Systemic Sclerosis Related Interstitial Lung Disease
Shervin Assassi¹, Ning Li ², Elizabeth Volkmann ³, Maureen Mayes ¹, Jun Ying ⁴, Michael Roth ⁵, Philip Clements ², Daniel Furst ⁶, Dinesh Khanna ⁷, Jonathan Goldin ², Robert Elashoff ² and Donald Tashkin ², ¹Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, Texas, USA, Houston, TX, ²University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ³University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, ⁴University of Texas McGovern Medical School at Houston, Houston, TX, ⁵University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁶University of California, Los Angeles, CA, ⁷Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor
Background/Purpose: Response to immunosuppression is highly variable in systemic sclerosis (SSc) related interstitial lung disease (ILD), and there are no widely accepted clinical or biological…

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