Abstracts tagged "Scleroderma"

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…
Abstract Number: 721 • 2019 ACR/ARP Annual Meeting
Ultrasound Measurement of the Nail Bed Matrix Thickness as a Useful Marker for Scleroderma-Related Interstitial Lung Disease
Marian Sidor¹, Roman Zuckerman ¹, Naomi Schlesinger ², Ross Sussman ³, Huma Jaleel Sabahath ⁴ and Vivien Hsu ⁵, ¹Rutgers-RWJ Medical School, New Brunswick, NJ, ²Rutgers Health- RWJ Medical School, New Brunswick, NJ, ³Rutgers-RWJ Medical School, East Brunswick, NJ 08816, NJ, ⁴AtlantiCare Regional Medical Center, Atlantic City, NJ, ⁵Rutgers- RWJ Medical School, SOUTH PLAINFIELD, NJ
Background/Purpose: Lung involvement is the leading cause of death in systemic sclerosis (SSc) (1). Pulmonary function tests (PFTs) and chest high-resolution CT scan (HRCT) are…
Abstract Number: 1062 • 2019 ACR/ARP Annual Meeting
Lymphocyte Subset Abnormalities in Early Diffuse Cutaneous Systemic Sclerosis
David Fox¹, Steven Lundy ², Michael Whitfield ³, Veronica Berrocal ⁴, Phillip Campbell ⁵, Stephanie Rasmussen ⁵, Ray Ohara ⁶, Alexander Stinson ⁶, Evan Wiewiora ⁷, Cathie Spino ⁷, Erica Bush ⁸, Daniel Furst ⁹, Shiv Pillai ¹⁰ and Dinesh Khanna ¹¹, ¹Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence,University of Michigan, Ann Arbor, MI, ²SystImmune, Inc., Redmond, WA, ³Department of Biomedical Data Science, Geisel School of Medicine at Dartmouth, Hannover, NH, ⁴Department of Biostatistics, School of Publich Health, University of Michigan, Ann Arbor, MI, ⁵Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, ⁶Washington University, St. Louis, MO, ⁷Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, ⁸Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, ⁹University of California, Los Angeles, CA, ¹⁰Ragon Institude of MGH, MIT and Harvard, Charlestown, MA, ¹¹Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence, University of Michigan, Ann Arbor
Background/Purpose: A variety of abnormalities in lymphocyte surface markers and functional subsets have been described in patients with systemic sclerosis (SSc), but conflicting results abound,…
Abstract Number: 1835 • 2019 ACR/ARP Annual Meeting
Frequency and Predictors of Meaningful Decline in Forced Vital Capacity During Follow up оf a Large Cohort of Systemic Sclerosis Associated Pulmonary Fibrosis Patients
Svetlana Nihtyanova¹, Emma Derrett-Smith ¹, Carmen Fonseca ¹, Voon Ong ² and Christopher Denton ³, ¹UCL Medical School, Royal Free Campus, London, United Kingdom, ²UCL Medical School, Royal Free Campus, London, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity…

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