Abstracts tagged "fibrosis and scleroderma"

Abstract Number: 116 • 2018 ACR/ARHP Annual Meeting
Identifying Matricellular Protein CYR61 As a Potential Anti-Fibrotic and Pro-Angiogenic Mediator in Scleroderma
Pei-Suen Tsou¹, Dinesh Khanna² and Amr H Sawalha¹, ¹Division of Rheumatology, University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, University of Michigan Scleroderma Program, Ann Arbor, MI
Background/Purpose: Vascular abnormalities represent a fundamental event in the pathogenesis of scleroderma (SSc) in that endothelial cell (EC) damage triggers a self-fueling process ending in…
Abstract Number: 131 • 2018 ACR/ARHP Annual Meeting
Myofibroblast Cells Expression of CD248 May Contribute to Exacerbate Microvascular Damage during Systemic Sclerosis
Paola Di Benedetto¹, Rebecca Ross², Paola Cipriani¹, Filomena Esteves³, Vasiliki Liakouli¹, Piero Ruscitti¹, Francesco Carubbi¹, Onorina Berardicurti¹, Noemi Panzera¹, Salvatore Di Bartolomeo¹, Chiara Galloni⁴, Georgia Mavria⁴, Francesco Del Galdo² and Roberto Giacomelli¹, ¹Department of Applied Clinical Sciences and Biotechnology, Rheumatology Unit, University of L'Aquila, L'Aquila, Italy, ²Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ³Leeds Institute of Cancer and Pathology, University of Leeds, Leeds, United Kingdom, ⁴Signal Transduction and Tumour Microenvironment Group, Leeds Institute of Cancer and Pathology, University of Leeds, Leeds, Italy
Background/Purpose: Microvascular rarefaction and tissue fibrosis are the hallmarks of Systemic Sclerosis (SSc). CD248, also known as endosialin, is a transmembrane glycoprotein expressed on key…
Abstract Number: 1717 • 2018 ACR/ARHP Annual Meeting
Expert Consensus on the Screening, Treatment, and Management of Patients with Systemic Sclerosis-Interstitial Lung Disease, and the Potential Role of Anti-Fibrotics in a Treatment Paradigm for Systemic Sclerosis-Interstitial Lung Disease: A Delphi Consensus Study
Dinesh Khanna^1,2, Mary Strek³, Brian Southern⁴, Rajan Saggar⁵, Vivien Hsu⁶, Maureen D. Mayes⁷, Rick Silver⁸, Virginia D. Steen⁹, Donald Zoz¹⁰ and Franck Rahaghi¹¹, ¹Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, University of Michigan, Ann Arbor, MI, ³Pulmonology, University of Chicago, Chicago, IL, ⁴Pulmonology, Cleveland Clinic Ohio, Cleavland, OH, ⁵Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁶Rheumatology, Robert Wood Johnson University Scleroderma Program, New Brunswick, NJ, ⁷Rheumatology, University of Texas Medical School at Houston, Houston, TX, ⁸Rheumatology, Medical University of SC, Charleston, SC, ⁹Dept of Rheumatology, Georgetown Univ Medical Center, Washington, DC, ¹⁰Pulmonology, Boehringer Ingelheim, Ridgefield, CT, ¹¹Cleveland Clinic Florida Weston, Weston, FL
Background/Purpose: Systemic sclerosis (SSc), or scleroderma, is a rare, autoimmune, multisystem connective tissue disorder characterized by vascular damage, autoimmunity, and fibrosis of the skin and…
Abstract Number: 767 • 2017 ACR/ARHP Annual Meeting
Elevated MeCP2 Expression in Diffuse Cutaneous Systemic Sclerosis Dermal Fibroblasts Is Associated with Anti-Fibrotic Effects
Ye He¹, Pei-Suen Tsou², Dinesh Khanna³ and Amr H Sawalha², ¹Rheumatology, University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, University of Michigan, Ann Arbor, MI, ³University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic Sclerosis (SSc) is a multisystem autoimmune connective tissue disorder characterized by vascular injury and fibrosis of the skin and internal organs. Methyl-CpG-binding protein…
Abstract Number: 776 • 2017 ACR/ARHP Annual Meeting
Circulating Fibrocytes in Systemic Sclerosis Patients and Healthy Subjects: An in Vitro Study
Maurizio Cutolo¹, Paola Montagna², Stefano Soldano², Amelia Chiara Trombetta³, Paola Contini⁴, Vanessa Smith⁵, Barbara Ruaro², Alberto Sulli³ and Renata Brizzolara², ¹Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy, Genoa, Italy, ²Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino, Genoa, Italy, Genoa, Italy, ³Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS A.O.U. San Martino-IST, University of Genova, Genoa, Italy, Genova, Italy, ⁴Division of Clinical Immunology, Department of Internal Medicine, University of Genova, Genoa, Italy, Genova, Italy, ⁵Faculty of Internal Medicine, Ghent University, Ghent, Belgium
Background/Purpose: Circulating fibrocytes seem to exert immunomodulatory effects, expressing class II major histocompatability complex molecules (HLA-DP, -DQ, and -DR) and could have a role in…
Abstract Number: 806 • 2016 ACR/ARHP Annual Meeting
Single Cell Rnaseq Defines a Unique Transcriptome Profile for Myofibroblasts in the Skin of Patients with Systemic Sclerosis
Robert A. Lafyatis¹, Lisa Rice², Giuseppina Stifano², Jeff Browning^2,3 and Robert W. Simms⁴, ¹Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ²Boston University School of Medicine, Boston, MA, ³Boston university, Cambridge, MA, ⁴Rheumatology, Boston University School of Medicine, Boston, MA
Background/Purpose: Fibrosis in systemic sclerosis (SSc), as well as a wide variety of other fibrotic diseases, is largely driven by myofibroblasts. Myofibroblasts are known to…
Abstract Number: 1855 • 2016 ACR/ARHP Annual Meeting
Phosphodiesterase-5 Inhibitors Attenuate Fibrotic Phenotype and Restore Anti-Fibrotic Resopnses of Cutaneous Fibroblasts in Patients with Scleroderma
Vikas Agarwal¹, Mohit kumar Rai¹, Vinita Agrawal², Harshit Singh¹ and Saurabh Chaturvedi¹, ¹Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, ²Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Lucknow, India
Background/Purpose: Scleroderma (SSc) is a chronic autoimmune disease, characterized by excessive fibrosis of skin and internal organs due to uncontrolled proliferation of fibroblasts and deposition…
Abstract Number: 1856 • 2016 ACR/ARHP Annual Meeting
Discovery of a Small Molecule Inhibitor of the Wnt Pathway (SM04755) As a Potential Topical Treatment for Scleroderma
Vishal Deshmukh¹, Allison Hood², Maureen Ibanez¹, Luis Dellamary¹, Josh Stewart¹, Timothy Seo¹, John Hood² and Yusuf Yazici¹, ¹Samumed, LLC, San Diego, CA, ²Samumed, LLC (formerly), San Diego, CA
Background/Purpose: Scleroderma is an autoimmune fibrotic disease, which presents skin manifestations among others. The Wnt pathway plays an important role in inflammation, skin fibrosis, and…
Abstract Number: 3264 • 2015 ACR/ARHP Annual Meeting
Multiplex Serum Protein Analysis in Systemic Sclerosis Defines Potential Anti-Fibrotic Mechanisms and Markers of Response to Hyperimmune Caprine Serum
Niamh Quillinan¹, Kristina E.N. Clark¹, Deirdre McIntosh², Jeffrey Vernes² and Christopher P. Denton³, ¹Rheumatology, UCL Division of Medicine, London, United Kingdom, ²Daval International, London, United Kingdom, ³Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom
Background/Purpose: Hyperimmune caprine serum (HICS) administered by subcutaneous injection over 26 weeks has shown benefit for modified Rodnan skin score in diffuse systemic sclerosis…
Abstract Number: 771 • 2014 ACR/ARHP Annual Meeting
Development of a Bifluorescent Lineage Tracker Reporter Mouse Strain to Analyze the Phenotypic Conversion of Endothelial Cells into Myofibroblasts in Vivo. Application to Study the Synergistic Effects of Endothelin-1 on TGF-β1-Induced Endothelial-to-Mesenchymal Transition
Peter J. Wermuth and Sergio A. Jimenez, Jefferson Institute of Molecular Medicine, Division of Connective Tissue Diseases and Scleroderma Center,Thomas Jefferson University, Philadelphia, PA
Background/Purpose: Endothelial-to-mesenchymal transition (EndoMT) may be a crucial pathway in generating activated myofibroblasts, cells that play a pivotal role in the development of tissue and…
Abstract Number: 1796 • 2014 ACR/ARHP Annual Meeting
Autotaxin Is Highly Expressed in Systemic Sclerosis (SSc) Skin, Mediates Dermal Fibrosis Via IL-6, and Is a Target for SSc Therapy
Flavia V. Castelino^1,2, Leaya M. George³, Gretchen Bain⁴, Lance Goulet⁵, Robert Lafyatis⁶ and Andrew M. Tager^3,7, ¹Rheumatology, Massachusetts General Hospital, Boston, MA, ²Harvard Medical School, Boston, MA, ³Pulmonary Unit, Massachusetts General Hospital, Boston, MA, ⁴Biology, PharmAkea Pharmaceuticals, San Diego, CA, ⁵PharmAkea Pharmaceuticals, San Diego, CA, ⁶Rheumatology, Boston University School of Medicine, Boston, MA, ⁷Massachusetts General Hospital, Boston, MA
Background/Purpose Autotaxin (ATX) is an enzyme present in biological fluids that is responsible for the production of the lipid mediator, lysophosphatidic acid (LPA). We previously…
Abstract Number: 1725 • 2014 ACR/ARHP Annual Meeting
The SYK Inhibitor Fostamatinib Limits Tissue Damage and Fibrosis in a Bleomycin-Induced Scleroderma MOUSE MODEL
Omer Nuri Pamuk¹, Guray Can², Suleyman Ayvaz³, Turan Karaca⁴, Gulsum Pamuk⁵, Selim Demirtas⁶ and George C. Tsokos⁷, ¹Rheumatology, Trakya University Medical Faculty, Edirne, Turkey, ²Gastroenterology, Trakya University Medical Faculty, Edirne, Turkey, ³Pediatric Surgery, Trakya University Medical Faculty, Edirne, Turkey, ⁴Histology, Trakya University Medical Faculty, Edirne, Turkey, ⁵Hematology, Trakya University Medical Faculty, Edirne, Turkey, ⁶Trakya University Medical Faculty, Edirne, Turkey, ⁷Rheumatology, Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, MA
Background/Purpose The possible antifibrotic effects of various kinase inhibitors has been studied before in SSc. Spleen tyrosine kinase (Syk) is a protein tyrosine kinase which…
Abstract Number: 969 • 2014 ACR/ARHP Annual Meeting
Caspase-8 Prevents Lung Fibrosis in a Murine SSc-like Disease Model By Preventing Macrophage Differentiation
Alexander Misharin¹, Carla M. Cuda², Luisa Morales-Nebreda³, Gokhan Mutlu¹, GR Scott Budinger³ and Harris R. Perlman⁴, ¹Northwestern University, Chicago, IL, ²Medicine / Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, ³Medicine, Northwestern University, Feinberg School of Medicine, Chicago, IL, ⁴Northwestern University Feinberg School of Medicine, Chicago, IL
Background/Purpose Pulmonary fibrosis has emerged as the leading cause of death in patients with Systemic Sclerosis (SSc). Currently available therapies are only marginally effective in…
Abstract Number: 964 • 2014 ACR/ARHP Annual Meeting
Skin Collagen Synthesis Rates Distinguish Between Early and Late Diffuse Scleroderma Patients
Claire Emson¹, Martin Decaris¹, Michelle Gatmaitan¹, Flora Luo¹, Dan Holochwost¹, Simplicia FloraCruz¹, Thomas Angel¹, Kelvin Li¹, Marc Hellerstein¹, Fredrick M. Wigley², Scott Turner^1,3 and Francesco Boin², ¹KineMed Inc., Emeryville, CA, ²Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, ³5980 Horton Street, Suite 470, KineMed Inc., Emeryville, CA
Background/Purpose The synthesis and degradation of extracellular matrix (ECM), particularly collagen, is one of the central mechanisms perturbed in scleroderma (SSc). Understanding the kinetics of…

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