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  • Abstract Number: 271 • 2016 ACR/ARHP Annual Meeting

    Impaired Satellite Cell Activation and Myofiber Transition during Skeletal Muscle Regeneration in Patients with Polymyositis and Dermatomyositis

    Beatriz Hanaoka1, Prabhakara R. Nagareddy2, Marilyn Campbell3, Leslie J. Crofford4, Charlotte A. Peterson5, Lisa G. Rider6 and Frederick W. Miller7, 1Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 2Nutritional Sciences, University of Alabama at Birmingham, Birmingham, AL, 3University of Kentucky, Lexington, KY, 4Medicine, Vanderbilt University Medical Center, Nasville, TN, 5College of Health Sciences, University of Kentucky, Lexington, KY, 6Rheumatology, George Washington University, Washington, DC, 7Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, NIH, Bethesda, MD

    Background/Purpose: Satellite cells are myogenic stem cells that are essential for myofiber repair, growth and homeostasis in postnatal life. A decline in satellite cell performance…
  • Abstract Number: 272 • 2016 ACR/ARHP Annual Meeting

    Interferon Chemokine Score and Other Cytokine Measures Predict Changes in Disease Activity in Patients with Juvenile and Adult Dermatomyositis

    Cynthia S. Crowson1, Jeannette M. Olazagasti Lourido2, Molly S. Hein3, Richard S. Pendegraft4, Michael A. Strausbauch5, Timothy B. Niewold6, Floranne C. Ernste7, Theresa L. Wampler Muskardin3, Erik J. Peterson8, Emily C. Gillespie9 and Ann M Reed10, 1Health Sciences Research, Mayo Clinic, Rochester, MN, 2University of Puerto Rico, San Juan, Puerto Rico, 3Rheumatology, Mayo Clinic, Rochester, MN, 4Biomedical Statistics and informatics, Rochester, MN, 5Surgical Research, Mayo Clinic, Rochester, MN, 6Rheumatology and Immunology, Mayo Clinic, Rochester, MN, 7Division of Rheumatology, Mayo Clinic Rochester, Rochester, MN, 8Center for Immunology/Department of Medicine, University of Minnesota, Minneapolis, MN, 9Medicine, University of Minnesota, Minneapolis, MN, 10Rheumatology, Duke University, Durham, NC

    Background/Purpose:   Methods:  Multiplexed immunoassays (Meso Scale Discovery) enabled simultaneous measurement of IFN-regulated chemokines and other pro- and anti-inflammatory cytokines specific to differentiation of specific…
  • Abstract Number: 273 • 2016 ACR/ARHP Annual Meeting

    Juvenile Dermatomyositis Patient-Derived Induced Pluripotent Stem Cells Do Not Retain Disease Expression Signatures

    Elisha D.O. Roberson1,2, Li Cao1, David J. Morales-Heil1, Dong Xu3,4, Yekaterina Galat5, Vasiliy Galat5,6, Stacey Tarvin7, Chiang-Ching Huang8 and Lauren M. Pachman4,9, 1Department of Medicine, Washington University, St. Louis, MO, 2Department of Genetics, Washington University, St. Louis, MO, 3Program of Excellence in Cure-Juvenile Myositis (JM) Research, Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 4Division of Pediatric Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 5Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 6Department of Pathology, Northwestern University, Chicago, IL, 7Riley Hospital for Children, Indiana University, Indianapolis, IN, 8Zilber School of Public Health, University of Wisconsin at Milwaukee, Milwaukee, WI, 9Cure JM Program of Excellence in Juvenile Myositis Research, Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL

    Background/Purpose: Monozygotic twins discordant for JDM are rare, composing only 1% of our entire registry of 525 JM patients. These individuals represent a unique opportunity…
  • Abstract Number: 274 • 2016 ACR/ARHP Annual Meeting

    Splicing Factor Proline/Glutamine-Rich Is a Novel Autoantigen of Dermatomyositis and Associated with Anti-Melanoma Differentiation-Associated Gene 5 Antibody.

    Yuji Hosono1, Ran Nakashima1, Kosaku Murakami1, Yoshitaka Imura1, Satoshi Serada2, Minoru Fujimoto3, Hajime Yoshifuji1, Koichiro Ohmura4, Tetsuji Naka5 and Tsuneyo Mimori4, 1Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, 2Laboratory for Immune Signal, National Institute of Biomedical Innovation, Health and Nutrition, Ibaraki, Japan, 3Laboratry of immune signal, National Institute of Biomedical Innovation, Health and Nutrition, Ibaraki, Japan, 4Kyoto University Graduate School of Medicine, Kyoto, Japan, 5Laboratory for immune signal, National Institute of Biomedical Innovation, Health and Nutrition, Ibaraki, Japan

    Background/Purpose:   Anti- melanoma differentiation-associated gene 5 (MDA5) antibody positive dermatomyositis (DM) and clinically amyopathic DM (CADM) often develop rapidly progressive interstitial lung disesase (RP-ILD),…
  • Abstract Number: 275 • 2016 ACR/ARHP Annual Meeting

    In Vitro Activation of Type I Interferon Pathway Reproduces the Characteristics Damages Observed in Dermatomyositis Patients

    Leandro Ladislau1,2,3, Xavier Suárez-Calvet1,4, Claudia Benjamin3, Ségolène Toquet1, Benjamin Terrier5, Flore Rozenberg6, Vincent Mouly1, Gillian Butler Browne7, Werner Stenzel8, Olivier Benveniste9 and Yves Allenbach10, 1Sorbonne Universités, UPMC Univ Paris 06, INSERM UMRS_974, CNRS FRE 3617, Center of Research in Myology., Paris, France, 2Programa de Ciências Biomédicas, Instituto de Ciências Biomédicas, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 3Instituto de Biofísica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 4Neuromuscular Diseases Unit, Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona and Institut de Recerca Sant Pau., Barcelona, Spain, 5Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 6Departement de Virologie, Hôpital Cochin, Paris Descartes Universités, Paris, France, 7Sorbonne Universités UPMC Univ Paris 06, Myology research center, INSERM UMRS974, CNRS FRE3617, Pitié-Salpêtrière University Hospital, Paris, France, Paris, France, 8Charité-Universitätsmedizin Berlin, Berlin, Germany, 9Pitié-Salpêtrière University Hospital, Paris, France, 10Internal Medicine, Pitié-Salpêtrière University Hospital, Paris, France

    Background/Purpose: The type I interferons (IFN-I) including IFN-a, and IFN-b are key cytokines involved in innate immune response to viral infection. Almost all cells can…
  • Abstract Number: 276 • 2016 ACR/ARHP Annual Meeting

    Histological Study on the Expression of Transcriptional Intermediary Factor 1 (TIF1) in the Patients with Idiopathic Inflammatory Myopathies

    Young Kim1, Jinhyun Kim2, Seong-Wook Kang3, Seung Cheol Shim2, In-Seol Yoo2, Su-Jin Yoo1 and Sung Hae Chang4, 1Rheumatology, Daejeon Rheumatoid & Degenerative Arthritis Center, Chungnam National University Hospital, Daejeon, South Korea, 2Division of Rheumatology, Department of Internal Medicine, Daejeon Rheumatoid & Degenerative Arthritis Center, Chungnam National University Hospital, Daejeon, Korea, The Republic of, 3Daejeon Rheumatoid & Degenerative Arthritis Center, Chungnam National University Hospital, Gwangju, South Korea, 4Internal Medicine, Rheumatology, Soonchunhyang University, College of Medicine, Cheonan, South Korea

    Background/Purpose:  Myositis-specific antibodies in patients with inflammatory myopathies are known to be associated with various clinical manifestations, classifications and diagnosis. Among them, recently found anti-transcriptional…
  • Abstract Number: 277 • 2016 ACR/ARHP Annual Meeting

    Abnormal Composition of Circulating T and B Cells in Patients with Polymyositis and Dermatomyositis Is More Biased in Those with Interstitial Lung Diseases

    Hirokazu Sasaki, Akito Takamura, Kimito Kawahata and Hitoshi Kohsaka, Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan

    Background/Purpose: Polymyositis (PM) and dermatomyositis (DM) are systemic inflammatory myopathies. They sometimes accompany interstitial lung disease (ILD), which can lead often to fatal outcome. For…
  • Abstract Number: 278 • 2016 ACR/ARHP Annual Meeting

    Expression of Anti-Microbial Peptide LL-37 Correlates to the Activation of Type I Interferon Pathway in Patients with Idiopathic Inflammatory Myopathies

    Xin Lu1, Quan Tang2, Monica Lindh3, Birgitta Agerberth4, Maryam Dastmalchi5, Ingrid E. Lundberg6 and Cecilia Wick7, 1Rheumatology, China-Japan Friendship Hospital, Beijing, China, 2Department of Medicine, Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden, 3Department of Laboratory Medicine, Division of Clinical Microbiology, Karolinska Institutet, Karolinska University Hospital Huddinge, Stockholm, Sweden, 4Department of Medical Biochemistry and Biophysics, Chemistry I, Karolinska Institute, Stockholm, Sweden, Stockholm, Sweden, 5Unit of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden, 6Department of Medicine, Rheumatology Unit, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden, 7Rheumatology Unit, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden

    Background/Purpose: Polymyositis (PM) and dermatomyositis (DM) are systemic autoimmune diseases whose pathogenesis remain unclear. The type I interferon system has recently been suggested to have…
  • Abstract Number: 279 • 2016 ACR/ARHP Annual Meeting

    Increased Heat Shock Protein 90 in Muscle Tissue and Plasma in Idiopathic Inflammatory Myopathies Correlates with Disease Activity and Skeletal Muscle Involvement

    Hana Storkanova1, Olga Krystufkova1, Martin Klein2, Herman F Mann1, Lucia Vernerova1, Maja Spiritovic1,3, Josef Zámecník4, Karel Pavelka2, Ladislav Senolt1, Jiří Vencovský1 and Michal Tomcik1, 1Institute of Rheumatology, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, 2Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University in Prague, Prague, Czech Republic, 3Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, 4Department of Pathology and Molecular Medicine, 2nd Medical School and University Hospital Motol, Charles University, Prague, Czech Republic

    Background/Purpose:  Heat shock proteins (Hsps) are chaperones playing important roles in skeletal muscle physiology, adaptation to exercise or stress, and activation of inflammatory cells. The…
  • Abstract Number: 280 • 2016 ACR/ARHP Annual Meeting

    Increased Expression of TIF-1 Gamma in Duodenal Adenocarcinoma from a Patient with p155/140 Dermatomyositis: A Causal Relationship?

    Marcello DiStasio1 and George Stojan2, 1Pathology, Beth Israel Deaconess Medical Center, Boston, MA, 2Division of Rheumatology, Beth Israel Deaconess Medical Center, Boston, MA

    Increased expression of TIF-1 gamma in duodenal adenocarcinoma from a patient with p155/140 dermatomyositis: a causal relationship? Background/Purpose: poor prognosis and worse survival in breast…
  • Abstract Number: 281 • 2016 ACR/ARHP Annual Meeting

    Characterization of Anti-3-Hydroxy-3-Methylglutaryl Coenzyme a Reductase Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies

    Takayuki Kishi1, Andrew Mammen2,3, Katherine Pak2, Lilliana Barillas-Arias4, Michael Henrickson5, Paul L. McCarthy6, Bracha Shaham7, Pamela F. Weiss8, Iren Horkayne-Szakaly9, Frederick W. Miller10, Lisa G. Rider10 and the Childhood Myositis Heterogeneity Study Group, 1Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, NIH, Bethesda, MD, 2National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 3Neurology and Medicine, Johns Hopkins University, Baltimore, MD, 4Bernard & Millie Duker Children's Hospital, Albany Medical Center, Albany, NY, 5Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 6Department of Pediatrics, Yale University School of Medicine, New Haven, CT, 7Pediatric Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA, 8Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 9Joint Pathology Center, Defense Health Agency, Silver Spring, MD, 10Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

    Background/Purpose:  Autoantibodies (Abs) to 3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase (HMGCR) identified in adult myositis patients with immune-mediated necrotizing myopathies (IMNM) have been associated with severe weakness,…
  • Abstract Number: 282 • 2016 ACR/ARHP Annual Meeting

    From ‘immune Mediated Necrotizing Myopathy’ to ‘antibody-Mediated Necrotizing Myositis: Towards the Pathogenic Role of Anti-SRP and Anti-Hmgcr Antibodies’

    Yves Allenbach1, Louiza Arouche-Delaperche2, Corinna Preusse3, Gillian Butler Browne2, Nicolas Champtiaux4, Kuberaka Mariampillai5, Aude Rigolet6, Peter Hufnagl7, Norman Zerbe8, Thierry Maisonobe9, Damien Amelin2, Sarah Leonard-louis10, Charles Duyckaerts11, Bruno Eymard12, Hans-Hilmar Goebel3, Laurent Drouot13, Olivier Boyer14, Olivier Benveniste2,5 and Werner Stenzel3, 1Pitié-Salpêtrière University Hospital, AP-HP, Department of Internal Medicine and Clinical Immunology, Paris, France, Paris, France, 2Sorbonne Universités UPMC Univ Paris 06, Myology research center, INSERM UMRS974, CNRS FRE3617, Pitié-Salpêtrière University Hospital, Paris, France, Paris, France, 3Charité - Universitätsmedizin, Department of Neuropathology, Berlin, Germany, Berlin, Germany, 4Department de Internal Medicine and Clinical Immunology, Hôpital Pitié-Salpêtrière, AP-PH, UPMC, Paris, France, 5Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Department of Internal Medicine and Clinical Immunology, Hospital University Department: inflammation, immunopathology and biotherapy (DHU i2B), Paris, France, Paris, France, 6Internal Medicine, Pitié-Salpêtrière University Hospital, Paris, France, 7Pathology departmen, Charite Hospital, Berlin, Germany, 8Department of Pathology, Charite Hospital, Berlin, Germany, 9Pitié-Salpêtrière University Hospital, AP-HP, Department of Neuropathology, Paris, France, Paris, France, 10Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Department of Neuropathology, Paris, France, Paris, France, 11Neuropathology, Pitie-Salpetriere Hospital, Paris, France, 12Department of Neurology, Hôpital Pitié-Salpêtrière, AP-PH, UPMC, Paris, France, 13Immunology, INSERM U905, University of Rouen, Rouen, France, 14Immunology, Inserm 905 & Institute for Biomedical Research, University of Rouen, Rouen, France

    Background/Purpose: Immune mediated necrotizing myopathy (IMNM) has been recently added as a new entity among dermatomyositis, polymyositis and sporadic inclusion body myositis. IMNM is defined…
  • Abstract Number: 283 • 2016 ACR/ARHP Annual Meeting

    Extracellular Histidyl-tRNA Synthetase in Myositis

    Catia Fernandes-Cerqueira1, Azita Sohrabian2, Inka Albrecht1, Antonella Notarnicola1, Elena Ossipova1, Johan Lengqvist1, Kim Kultima3, Maryam Fathi4, Ger JM Pruijn5, Johan Grunewald4, Johan Rönnelid2, Ingrid E. Lundberg1 and Per-Johan Jakobsson1, 1Department of Medicine, Rheumatology Unit, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden, 2Department of Immunology Genetics and Pathology,Uppsala University, Uppsala, Sweden, 3Department of Medical Sciences, Cancer Pharmacology and Computational Medicine, Uppsala University, Uppsala, Sweden, 4Department of Medicine, Division of Respiratory Medicine, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden, 5Biomolecular Chemistry, Institute for Molecules and Materials and Radboud Institute for Molecular Life Sciences, Radboud University, Nijmegen, Netherlands

    Background/Purpose: Histidyl-transfer RNA synthetase (HisRS, Jo-1) is a major autoantigen in myositis with lung involvement1-4. Simultaneous presence of anti-Jo-1 and anti-Ro52 antibodies has been demonstrated…
  • Abstract Number: 284 • 2016 ACR/ARHP Annual Meeting

    Gene-Environmental Interaction of HLA-DRB1*03:01 and Smoking for the Development of Anti-Jo-1 Autoantibodies in Idiopathic Inflammatory Myopathies: A UK Study

    Nicolas Pipis1, Simon Rothwell1, Robert Cooper2, Lucy R Wedderburn3, Neil J. McHugh4, Zoe Betteridge4, Janine Lamb5 and Hector Chinoy1,6, 1Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom, 2Department of Musculoskeletal Biology, University of Liverpool, Liverpool, United Kingdom, 3Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 4Department of Pharmacy and Pharmacology, University of Bath, Bath, United Kingdom, 5Centre for Integrated Genomic Medical Research, University of Manchester, Manchester, United Kingdom, 6NIHR Manchester Musculoskeletal Biomedical Research Unit, Manchester Academic Health Science Centre, Manchester, United Kingdom

    Background/Purpose: The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and extramuscular manifestations. The most common autoantibody…
  • Abstract Number: 285 • 2016 ACR/ARHP Annual Meeting

    Antibodies to Small Ubiquitin-like Modifier Activating Enzyme: Frequency and Characteristics of Antibody-Positive Patients in an Unselected Cohort

    Anne Tebo1, Troy Jaskowski2 and Lisa Peterson1, 1Pathology, University of Utah School of Medicine and ARUP Laboratories, Salt Lake City, UT, 2ARUP Laboratories, Salt Lake City, UT

    Background/Purpose:  Antibodies to small ubiquitin-like modifier activating enzyme (SAE) are associated with diagnosis of dermatomyositis (DM). The aims of this study were to establish the…
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