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  • Abstract Number: 1702 • 2017 ACR/ARHP Annual Meeting

    Disease Progression in Systemic Sclerosis Patients with Concomittant or Isolated Interstitial Lung Disease and Pulmonary Arterial Hypertension in the Scleroderma Cohort Singapore

    Maria Noviani1, Seyed Ehsan Saffari2, Sandra Mei Yu Kua1, Grace Yin Lai Chan3, Gim Gee Teng4, Weng Giap Law5, Amelia Santosa4, Anita Yee Nah Lim4, Swee Cheng Ng1 and Andrea Hsiu Ling Low1, 1Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore, Singapore, 2Center for Quantitative Medicine, Duke-NUS Medical School, Singapore, Singapore, Singapore, 3Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, SIngapore, Singapore, 4Division of Rheumatology, University Medicine Cluster, National University Health System, Singapore, Singapore, Singapore, 5Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore, Singapore

    Background/Purpose: Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are leading causes of mortality in patients with systemic sclerosis (SSc).  We aimed to determine…
  • Abstract Number: 1703 • 2017 ACR/ARHP Annual Meeting

    A Double-Blind, Randomized, Placebo-Controlled, Dose-Escalation, Multi-Center Study of a Single Intravenous Infusion of Allogeneic Mesenchymal Precursor Cells in Patients with Rheumatoid Arthritis and Incomplete Response to at Least One Tnfα Inhibitor

    Suzanne Kafaja1, Donna Skerrett2, Silviu Itescu3 and Daniel E. Furst4, 1Department of Internal Medicine, University of California Los Angeles, David Geffen School of Medicine, Division of Rheumatology, Los Angeles, CA, 2Mesoblast Inc., New York, NY, 3mesoblast Inc., New York, NY, 4David Geffen School of Medicine at UCLA, Los Angeles, CA

    Background/Purpose: Allogeneic STRO-3 immunoselected mesenchymal precursor cells (MPCs) derived from bone marrow of healthy donors are a potent, homogeneous cell population which can be activated…
  • Abstract Number: 1704 • 2017 ACR/ARHP Annual Meeting

    Non-Randomized Controlled Trial to Evaluate the Effect of Extracorporeal Shock Wave Therapy on Digital Ulcers in Systemic Sclerosis

    Tomonori Ishii1, Yasushi Kawaguchi2, osamu ishikawa3, naruhiko takasaawa4, takao kodera5, hidekata yasuoka6, yuichi takahashi7, osamu takai8, Izaya Nakaya9, Hiroshi Fujii10, Yukiko Kamogawa10, Yuko Shirota10, Tsuyoshi Shirai10, Yoko Fujita11, shinichiro saito12, Hiroaki Shimokawa13 and Hideo Harigae10, 1Clinical Research, Innovation and Education Center, Tohoku University Hospital, Sendai, Japan, 2Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, 3Department of Dermatology, Gunma University Graduate School of Medicine, gunma, Japan, 4Department of Internal Medicine, Tohoku Medical and Pharmaceutical University Wakabayashi Hospital, Sendai, Japan, 5Division of Hematology and Rheumatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan, 6Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, tokyo, Japan, 7Yu Family Clinic, Sendai, Japan, 8Osaki Citizen Hospital, Sendai, Japan, 9Department of Nephrology and Rheumatology, Iwate Prefectural Central Hospital, Morioka, Japan, 10Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan, 11Department of Hematolgy and Rheumatolgy, Tohoku University Graduate School of Medicine, Sendai, Japan, 12IMS Meirikai Sendai General Hospital, Sendai, Japan, 13Department of Cardiovascular medicine, Tohoku University Graduate School of Medicine, Sendai, Japan

    Background/Purpose: Patients with systemic sclerosis (SSc) often display Raynaud’s phenomenon, which causes digital skin ulcers. Since these ulcers are not associated with autoimmune factors, conventional…
  • Abstract Number: 1705 • 2017 ACR/ARHP Annual Meeting

    Inhibition of EZH2 Stops Fibrosis and Improves Angiogenesis in Scleroderma

    Pei-Suen Tsou1, Phillip L. Campbell2, M. Asif Amin3, Patrick Coit1, David Fox4, Dinesh Khanna5 and Amr H Sawalha1, 1Division of Rheumatology, University of Michigan, Ann Arbor, MI, 2Rheumatology, Division of Rheumatology, University of Michigan Medical Center, Ann Arbor, MI, 3Division of Rheumatology and Clinical Autoimmune Center of Excellence, University of Michigan, Ann Arbor, MI, Ann Arbor, MI, 4Department of Medicine [Division of Rheumatology], University of Michigan Medical System, Ann Arbor, MI, 5University of Michigan, Ann Arbor, MI

    Background/Purpose: Scleroderma (SSc) is a complex disease that involves activation of the immune system, vascular complications, and tissue fibrosis. Although the pathogenesis of this disease…
  • Abstract Number: 1706 • 2017 ACR/ARHP Annual Meeting

    Dipeptidyl-Peptidase-4 (DPP4) Promotes Fibroblast Activation and Is a Potential Molecular Target for Treatment of Fibrosis

    Alina Soare1, Hermina Györfy1, Alexandru Matei1, Clara Dees1, Chih-Wei Chen1, Andreas Ramming2, Georg Schett3 and Jörg Distler1, 1Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 2Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 3Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany

    Background/Purpose: Dipeptidyl-peptidase-4 (DPP4) has been reported to identify a dermal fibroblast lineage involved in scaring and its inhibition leads to reduced scar formation. Its role…
  • Abstract Number: 1707 • 2017 ACR/ARHP Annual Meeting

    Effect of Anabasum (JBT-101) on Gene Expression in Skin Biopsies from Subjects with Diffuse Cutaneous Systemic Sclerosis (dcSSc) and the Relationship of Baseline Molecular Subsets to Clinical Benefit in the Phase 2 Trial

    Viktor Martyanov1, Yolanda Nesbeth2, Guoshuai Cai1, Tammara A. Wood1, Jake Reder2, Scott Constantine3, Barbara White3, Robert F. Spiera4 and Michael L. Whitfield1, 1Department of Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, 2Celdara Medical, LLC, Lebanon, NH, 3Corbus Pharmaceuticals, Inc., Norwood, MA, 4Rheumatology, Hospital for Special Surgery, New York, NY

    Background/Purpose: Anabasum (JBT-101) is a non-immunosuppressive, synthetic, CB2 agonist that resolves inflammation and fibrosis in animal models of SSc and reduces TGF-β and collagen production…
  • Abstract Number: 1708 • 2017 ACR/ARHP Annual Meeting

    Molecular Imaging Biomarkers for Personalized Medicine Strategies in Systemic Sclerosis-Related Interstitial Lung Disease

    Janine Schniering1, Martina Benesova2,3, Matthias Brunner4, Carol A. Feghali-Bostwick5, Roger Schibli2,3, Oliver Distler6, Cristina Müller2,3 and Britta Maurer6, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland, Zurich, Switzerland, 2Department of Chemistry and Applied Biosciences, Department of Chemistry and Applied Biosciences, Swiss Federal Institute of Technology, Zurich, Switzerland, Zurich, Switzerland, 3Center for Radiopharmaceutical Sciences ETH-PSI-USZ, Center for Radiopharmaceutical Sciences ETH-PSI-USZ, Paul Scherrer Institute, Villigen-PSI, Switzerland, Villigen PSI, Switzerland, 4Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland, Zurich, Switzerland, 5Division of Rheumatology and Immunology, Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, United States, Charleston, SC, 6Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland

    Background/Purpose: Interstitial lung disease (ILD) is a life-threatening complication in SSc. In recent years, distinct genomic and molecular subtypes in SSc-ILD were identified and molecular…
  • Abstract Number: 1709 • 2017 ACR/ARHP Annual Meeting

    Classical Monocytes in the Pathogenesis of Early Diffuse Cutaneous Systemic Sclerosis

    Julia Dunn1, Salina Dominguez1, Philip J. Homan1, Carla Cuda1, Dinesh Khanna2, Shervin Assassi3, Tracy M. Frech4, Harris Perlman5, Deborah R. WInter1 and Monique Hinchcliff6, 1Department of Medicine Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 2University of Michigan, Ann Arbor, MI, 3University of Texas McGovern Medical School, Houston, TX, 4Division of Rheumatology, University of Utah, Salt Lake City, UT, 5Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Northwestern University Feinberg School of Medicine,, Chicago, IL, 6Northwestern University Institute for Public Health and Medicine, Chicago, IL

    Background/Purpose: In light of heterogeneous clinical manifestations in systemic sclerosis (SSc), transcriptional analysis of fibrotic tissue and circulating leukocytes may illuminate conserved processes driving inflammation…
  • Abstract Number: 1710 • 2017 ACR/ARHP Annual Meeting

    Down-Regulation of microRNA-126 in Scleroderma Microvascular Endothelial Cells Enhances the Transition of Endothelial to Mesenchymal Cells (Endo-MT) Induced By TGFβ through Down-Regulating PI3/Akt Signaling Pathway By Targeting PIK3R2

    Yongqing Wang1, Shadia Nada2, Nezam Altorok2 and Bashar Kahaleh2, 1University of Toledo, Toledo, OH, 2Medicine/Rheumatology, University of Toledo, Toledo, OH

    Background/Purpose: The accumulation of myofibroblasts in fibrotic tissue plays an important role in the pathogenesis of scleroderma (SSc) fibrosis. Recent studies have shown that myofibroblasts…
  • Abstract Number: 1711 • 2017 ACR/ARHP Annual Meeting

    SIRT1 May Protect Against Systemic Sclerosis-Related Pulmonary Fibrosis By Decreasing Pro-Inflammatory and Pro-Fibrotic Processes

    Haiyan Chu1, Shuai Jiang2, Qingmei Liu3, Feng Qian4, Xiaodong Zhou5, Maureen D. Mayes6, Li Jin7 and Jiucun Wang8, 1MOE Key Laboratory of Contemporary Anthropology, State Key Laboratory of Genetic Engineering and Ministry of Education Key Laboratory of Contemporary Anthropology, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, China, 2State Key Laboratory of Genetic Engineering and Ministry of Education Key Laboratory of Contemporary Anthropology, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, China, 3State Key Laboratory of Genetic Engineering and Ministry of Education Key Laboratory of Contemporary Anthropology, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, Chile, 4Ministry of Education Key Laboratory of Contemporary Anthropology, School of Life Sciences, Fudan University, Shanghai, China, 5Internal Medicine-Rheumatology, University of Texas McGovern Medical School, Houston, TX, 6University of Texas McGovern Medical School, Houston, TX, 7State Key Laboratory of Genetic Engineering, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, China, 8State Key Laboratory of Genetic Engineering, Collaborative Innovation Center for Genetics and Development, School of Life Sciences, Fudan University, Shanghai, CN

    Background/Purpose: Interstitial lung disease (ILD) is the leading cause of death in systemic sclerosis (SSc).  Sirtuin1 (SIRT1) is a deacetylase with known anti-inflammatory and anti-fibrotic…
  • Abstract Number: 1712 • 2017 ACR/ARHP Annual Meeting

    Microbial and Metabolic MULTI-Omic Correlations in Systemic Sclerosis Patients

    Chiara Bellocchi1, Alvaro Fernández-Ochoa2, Gaia Montanelli1, Barbara Vigone3, Alessandro Santaniello3, Christian Milani4, Rosa Quirantes-PIné2, Isabel Borras Linares2, Marco Ventura4, Antonio Segura Carretero2, Marta Alarcón-Riquelme5,6 and Lorenzo Beretta3, 1Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy, 2Department of Analytical Chemistry, University of Granada, Granada, Spain, 3Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 4Department of Life Sciences, Università degli studi di Parma, Parma, Italy, 5Centro de Genómica e Investigación Oncológica (GENYO), Pfizer-Universidad de Granada-Junta de Andalucía, Granada, Spain, 6Institute for Environmental Medicine, Karolinska Institutet, Stockholm, Sweden

    Background/Purpose: The gastro-intestinal tract (GIT) is frequently involved in Systemic sclerosis (SSc). Perturbation in the gut microbiota may affect the body well-being and function and…
  • Abstract Number: 1713 • 2017 ACR/ARHP Annual Meeting

    Defining Genetic Risk for Scleroderma Renal Crisis in RNA-Polymerase III Antibody Positive Patients

    Edward Stern1, Sandra Guerra1, Harry Chinque1, David Gonzalez Serna2, Markella Ponticos1, Javier Martin2, Maureen D. Mayes3, Shervin Assassi4, Carmen Fonseca1 and Christopher Denton5, 1UCL Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom, 2Instituto de Parasitología y Biomedicina López-Neyra, Granada, Spain, 3Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 4University of Texas McGovern Medical School, Houston, TX, 5Department of Rheumatology, University College London, Royal Free Hospital, London, United Kingdom

    Background/Purpose: Scleroderma renal crisis (SRC), characterised by accelerated hypertension and acute kidney injury, is a life-threatening complication of systemic sclerosis (SSc). Most SSc cases have…
  • Abstract Number: 1714 • 2017 ACR/ARHP Annual Meeting

    Inhibition of Hedgehog Acyltransferase Alleviates the Profibrotic Effects of Transforming Growth Factor β in Systemic Sclerosis

    Ruifang Liang1, Rosebeth Kagwiria2, Clara Dees3, Yun Zhang4, Oliver Distler5, Georg Schett6 and Jörg Distler7, 1Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 2Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 3Department of Internal Medicine 3 and Institute for Clinical Immunology,, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 4Department of Internal Medicine 3 and Institute for Clinical Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 5Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 6Department of Internal Medicine 3 – Rheumatology and Immunology, Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 7Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Background/Purpose: Hedgehog acyltransferase (Hhat) catalyzes the attachment of the fatty acid palmitate onto Sonic Hedgehog (Shh), a modification essential for Shh signaling activity. Palmitoylation of…
  • Abstract Number: 1715 • 2017 ACR/ARHP Annual Meeting

    Mitochondrial DNA Mutations and Respiratory Chain Dysfunction in Lung Fibrosis of Systemic Sclerosis

    Veronika K. Jaeger1, Dirk Lebrecht2,3, Andrew G. Nicholson4,5, Athol U Wells5,6, Suresh George7, Amiq Gazdhar8, Michael Tamm9, Nils Venhoff2, Thomas Geiser8 and Ulrich A. Walker1, 1Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 2Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany, 3Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany, 4Department of Histopathology, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom, 5National Heart and Lung Institute, Imperial College, London, United Kingdom, 6Interstitial Lung Disease Unit, Royal Brompton Hospital, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom, 7Royal Brompton and Harefield National Health Service Foundation Trust, London, United Kingdom, 8Department of Pulmonary Medicine, University Hospital Bern, Bern, Switzerland, 9Department of Pneumology, University Hospital Basel, Basel, Switzerland

    Background/Purpose: Recent data have implemented reactive oxygen species (ROS) in the etiology of interstitial lung disease (ILD) in systemic sclerosis. Recent data from bleomycin mice…
  • Abstract Number: 1716 • 2017 ACR/ARHP Annual Meeting

    TGFβ-Dependent Upregulation of XIAP Fosters Fibroblast Activation and Tissue Fibrosis By Promoting Canonical Wnt Signaling

    Christina Bergmann1, Ludwig Hallenberger1, Amelie Brandt1, Benita Merlevede1, Clara Dees2, Chih-Wei Chen3, Christian Beyer1, Oliver Distler4, Georg Schett5 and Jörg Distler6, 1Department of Internal Medicine 3 and Institute for Clinical Immunology, Friedrich-Alexander-University of Erlangen-Nuremberg and University Hospital Erlangen, Erlangen, Germany, 2Department of Internal Medicine 3 and Institute for Clinical Immunology,, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany, 3Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, 4Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 5Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, 6Department of Internal Medicine 3 – Rheumatology and Immunology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Erlangen, Germany

    Background/Purpose: Aberrant activation of profibrotic pathways is a key feature of systemic sclerosis (SSc). Extensive evidence characterizes TGFβ- and canonical WNT-signaling as key drivers of…
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