Abstracts Archive - Page 828 of 2425 - ACR Meeting Abstracts

Abstract Number: 1044 • 2019 ACR/ARP Annual Meeting
TGF-β Isoforms Modulate the RNA Cargo of Extracellular Vesicles (Exosomes) Isolated from Cultured Normal Human Lung Microvascular Endothelial Cells: A Mechanistic Link Between Endothelial Cell Dysfunction and the Establishment of a Profibrotic Phenotype in SSc?
Peter Wermuth¹ and Sergio Jimenez ¹, ¹Jefferson Institute of Molecular Medicine and Division of Connective Tissue Diseases, Thomas Jefferson University, Philadelphia, PA, Philadelphia, PA
Background/Purpose: Extracellular vesicles (EV) are a diverse assortment of lipid bilayer-bound vesicles of various sizes and origin. Exosomes are a subset of EV arising from…
Abstract Number: 1045 • 2019 ACR/ARP Annual Meeting
Myocardial Involvement in SSc: Key Role of Lin-gp38+ Stromal Cells in the Onset of Fibrosis and Defects of the Conduction System
Mara Stellato¹, Michal Rudnik ², Florian Renoux ³, Przemyslaw Blyszczuk ⁴, Elena Osto ⁵, Matthias Dewenter ⁶, Petra Seebeck ⁷, Elena Pachera ², Oliver Distler ⁸ and Gabriela Kania ⁴, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ²Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Zurich, Switzerland, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, ⁴Centre of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁵Institute of Clinical Chemistry, University Hospital Zurich, Switzerland, Zurich, Zurich, Switzerland, ⁶Department of Molecular Cardiology and Epigenetics, University Hospital, Heidelberg, Germany, Heidelberg, Germany, ⁷Zurich integrative Rodent Physiology, University of Zurich, Switzerland, Zurich, Switzerland, ⁸Dept. of Rheumatology, University Hospital Zürich, Zürich, Switzerland, Zürich, Switzerland
Background/Purpose: Cardiac fibrosis is a known complication of SSc, associated with increased mortality. During fibrogenesis, fibroblasts differentiate into myofibroblasts and secrete excessive extracellular matrix proteins.…
Abstract Number: 1046 • 2019 ACR/ARP Annual Meeting
The Effect of Nintedanib versus Mycopheolate Mofetil in the FRA2 Mouse Model of Systemic Sclerosis Associated Interstitial Lung Disease
Thuong Trin-Minh ¹, Yun Zhang ², Jörg Distler ³ and Lutz Wollin⁴, ¹University Erlangen-Nuremberg, Erlangen-Nuremburg, Germany, ²University Erlangen-Nuremberg, Erlangen, Germany, ³Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ⁴oehringer Ingelheim, Biberach, Germany
Background/Purpose: Interstitial lung disease (ILD) is a key driver of mortality in patients with systemic sclerosis (SSc). In lack of approved treatment it constitutes a…
Abstract Number: 1047 • 2019 ACR/ARP Annual Meeting
CXCL4-L1 Levels Are Elevated in Systemic Sclerosis Patients and Correlate with Pulmonary Arterial Hypertension and Capillaroscopic Indices of Vascular Damage
Vasiliki Kalliopi Bournia¹, Markos Patsouras ², Nikolaos Vlachoyiannis ³, Athanasios Tzioufas ⁴, Petros Sfikakis ⁵ and Panagiotis Vlachoyiannopoulos ⁶, ¹1st Department of Propaedeutic and Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ²Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Greece, Athens, ³1st Department of Propaedeutic and Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece, Athens, ⁴Department of Pathophysiology, Athens School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ⁵Joint Rheumatology Programme, National & Kapodistrian University of Athens Medical School, Athens, Greece, Athens, Greece, ⁶University General Hospital of Athens “Laiko”, Athens, Greece
Background/Purpose: Recent findings have implicated CXCL4 in the pathogenesis of systemic sclerosis (SSc), while also indicating a potential role of this chemokine as a biomarker…
Abstract Number: 1048 • 2019 ACR/ARP Annual Meeting
Clonally Expanded CD4+ Cytotoxic T Cells, Endothelial Cell Apoptosis and the Pathogenesis of Early Systemic Sclerosis
Takashi Maehara ¹, Cory Perugino², Naoki Kaneko ¹, Hamid Mattoo ³, Jesper Kers ⁴, Hugues Allard-Chamard ⁵, Vinay Mahajan ⁵, Hang Liu ⁵, Samuel Murphy ⁵, Musie Ghebremichael ⁵, Yesim Tuncay ⁵, Emanuel Della Torre ⁶, Lloyd Liang ², Sydney Montesi ⁷, Zachary Wallace ², David Fox ⁸, Robert Lafyatis ⁹, John Stone ¹⁰, Dinesh Khanna ¹¹ and Shiv Pillai ¹², ¹Kyushu University, Fukuoka, Japan, ²Massachusetts General Hospital, Boston, ³Sanofi, Cambridge, ⁴University of Amsterdam, Amsterdam, Netherlands, ⁵Ragon Institute of MGH, MTI and Harvard, Cambridge, ⁶Hospital San Raffaele, milan, Italy, ⁷Massachusetts General Hospital, Boston, MA, ⁸University of Michigan, Ann Arbor, ⁹University of Pittsburgh, Pittsburgh, PA, ¹⁰Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA, ¹¹Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor, ¹²Ragon Institude of MGH, MIT and Harvard, Charlestown, MA
Background/Purpose: The CD4+ T cell subset driving the pathogenesis of systemic sclerosis (SSc) remains poorly understood. Many different CD4+ T cell subsets have been implicated,…
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: 1050 • 2019 ACR/ARP Annual Meeting
Genome-Wide DNA Methylation Signatures in Classical Monocytes from African Ancestry Patients with Systemic Sclerosis
Peter Allen¹, Jena Wirth ², Nathan Wilson ², Jim Oates ³, Melissa Cunningham ², Devin Absher ⁴ and Paula Ramos ², ¹University of Alabama at Birmingham, Birmingham, AL, ²Medical University of South Carolina, Charleston, ³Division of Rheumatology & Immunology/Medical University of South Carolina, Charleston, SC, ⁴HudsonAlpha Institute for Biotechnology, Huntsville, AL
Background/Purpose: Systemic sclerosis (SSc) is a rare, fibrotic autoimmune disorder characterized by cutaneous and visceral fibrosis, immune dysregulation, and vasculopathy. It disproportionately affects individuals of…
Abstract Number: 1051 • 2019 ACR/ARP Annual Meeting
Identification of Differential Chromatin Accessibility Using ATAC-seq in a Novel 3D Tissue Culture System of Systemic Sclerosis
Tamar Wheeler¹, Ji-Qing Chen ¹, Diana Toledo ¹, Bhaven Mehta ², Yue Wang ², Meredith Brown ¹, Rajan Bhandari ³ and Mengqi Huang ⁴, ¹Dartmouth College, Hanover, ²Geisel School of Medicine at Dartmouth, Hanover, NH, ³Dartmouth College, Lebanon, ⁴Geisel School of Medicine at Dartmouth College, Hanover, NH
Background/Purpose: Although the majority of SSc research has focused on differential gene expression, recent studies havedemonstrated that non-coding epigenetic changes in chromatin accessibility are likely…
Abstract Number: 1052 • 2019 ACR/ARP Annual Meeting
The PPAR Agonist Lanifibranor Protects Against Right Ventricular Hypertrophy in a Mouse Model of Systemic Sclerosis Associated Pulmonary Hypertension
Emma Derrett-Smith ¹, Kristina Clark¹, Shiwen Xu ¹, David Abraham ¹, Olivier Lacombe ², Pierre Broqua ², Jean-Louis Junien ², Irena Konstantinova ² and Christopher Denton ³, ¹University College London, London, United Kingdom, ²Inventiva, Daix, France, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: The TβRIIΔk-fib transgenic mouse model of systemic sclerosis (SSc) develops constitutive structural vasculopathy with vessel wall fibrosis and is susceptible to pulmonary hypertension (PH)…
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: 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: 1055 • 2019 ACR/ARP Annual Meeting
Proteomic and Transcriptomic Analysis of Human Eosinophilic Fasciitis Fibroblasts
Benjamin Chaigne¹, Claire Fernandez ², Morgane le Gall ², Philippe Chafey ², Benjamin Saintpierre ², Virginie Salnot ², Francois Guillonneau ², Claire Le Jeunne ³ and Luc Mouthon ⁴, ¹Cochin University Hospital, Paris, France, ²Cochin Institute, Paris, France, ³Paris Descartes University, Cochin Hospital, Internal Medicine department, Paris, France, ⁴National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France
Background/Purpose: Eosinophilic fasciitis (EF) is a rare scleroderma-like disorder with less than 200 cases reported. Due to the rarity of the disease, data regarding its…
Abstract Number: 1056 • 2019 ACR/ARP Annual Meeting
Profibrotic Macrophage Activation in Systemic Sclerosis Is Dependent on the Mechanosensing MRTF-A Pathway
Tricia Lim¹, Kristina Krogmannova ¹, Shiwen Xu ¹, Bahja Ahmed Abdi ², David Abraham ¹, Christopher Denton ³ and Richard Stratton ¹, ¹University College London, London, United Kingdom, ²University College London, London, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, UK, London, United Kingdom
Background/Purpose: An M2-like alternative activation state of macrophages has been linked to the pathogenesis of several fibrotic disorders, including systemic sclerosis (SSc). MRTF-A is an…
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: 1058 • 2019 ACR/ARP Annual Meeting
Inhibition of Histone Readers Bromodomain and Extraterminal Domain Proteins Alleviates Scleroderma Fibrosis
Sirapa Vichaikul ¹, Phillip Campbell ², M. Asif Amin ³, Jeffrey H. Ruth ³, Dallas Rohraff ¹, David Fox ⁴, Dinesh Khanna ⁵, Amr Sawalha ⁶ and Pei-Suen Tsou³, ¹University of Michigan, Ann Arbor, ²Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, ³University of Michigan, Ann Arbor, MI, ⁴Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence,University of Michigan, Ann Arbor, MI, ⁵Scleroderma Program, Division of Rheumatology, Department of Internal Medicine, Autoimmunity Center of Excellence, University of Michigan, Ann Arbor, ⁶University of Pittsburgh & University of Michigan, Pittsburgh, PA
Background/Purpose: Epigenetic regulation, including histone acetylation, plays an important role in scleroderma (SSc) fibrosis. The binding of the bromodomain and extra-terminal domain proteins (BRDs) to…

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