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  • Abstract Number: 2417 • 2016 ACR/ARHP Annual Meeting

    High Dimensional Interrogation of the T Cell Immunome in Polyarticular Juvenile Idiopathic Arthritis Patients 

    Jing Yao Leong1, Justin Tiong2, Joo Guan Yeo2,3, Liyun Lai1, Phyllis Chen3, Loshinidevi D/O Thana Bathi3, Thaschawee Arkachaisri2, Daniel J Lovell4 and Salvatore Albani1,5, 1SingHealth Translational Immunology and Inflammation Centre, Singapore Health Services Pte Ltd, Singapore, Singapore, 2Rheumatology and Immunology, KK Women's and Children's Hospital, Singapore, Singapore, 3Singhealth Translational Immunology and Inflammation Centre, Singapore Health Services Pte Ltd, Singapore, Singapore, 4PRCSG Cincinnati Children's Hospital Medical Center, Cinncinnati, OH, 5Duke-National University of Singapore Medical School, Singapore, Singapore

    Background/Purpose: Clinical management of polyarticular JIA with anti-TNF-alpha has been met with moderate success, with up to 50% of patients demonstrating clinically meaningful efficacy. Concerns…
  • Abstract Number: 2418 • 2016 ACR/ARHP Annual Meeting

    Rnaseq Reveals Treatment-Associated Gene Expression Dynamics in Juvenile Idiopathic Arthritis CD4+ T Cells

    Kaiyu Jiang1, Lai Ping Wong1, Yanmin Chen1 and James Jarvis2, 1Pediatrics, University at Buffalo, Buffalo, NY, 2Pediatrics, SUNY Buffalo School of Medicine, Buffalo, NY

    Background/Purpose: While the field of pediatric rheumatology has made enviable strides in improving the lives of children with juvenile idiopathic arthritis (JIA), there is still…
  • Abstract Number: 2419 • 2016 ACR/ARHP Annual Meeting

    Single-Cell Analysis of CD163 mRNA and Protein Expression By Primeflow™ in Polarized Monocyte and Macrophage Populations

    Rachel Tan1, Sherry Thornton2, Alyssa Sproles2, Thuy Do3, Jonathan Schick4, Monica DeLay4 and Grant Schulert5, 1University of Cincinnati, Cincinnati, OH, 2Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, 3Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4Cincinnati Children's Hospital, Cincinnati, OH, 5Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

    Background/Purpose:  CD163 is involved in the regulation and resolution of innate inflammation and the removal of free hemoglobin from the blood via internalization of the…
  • Abstract Number: 2420 • 2016 ACR/ARHP Annual Meeting

    Investigating Genome-Wide Inbreeding Coefficients and Age of Diagnosis in a Multi-Ethnic Population of Childhood-Onset Systemic Lupus Erythematosus (cSLE)

    Chen Di Liao1, Deanna Morra1, Daniela Dominguez1, Shazia Ali1, Deborah M. Levy2, Earl Silverman3, Andrew Paterson1 and Linda T Hiraki1, 1The Hospital for Sick Children, Toronto, ON, Canada, 2Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 3University of Toronto, Toronto, ON, Canada

    Background/Purpose: Genetics plays an important role in the pathogenesis of systemic lupus erythematosus (SLE). Up to 20% of those affected with SLE are diagnosed in…
  • Abstract Number: 2421 • 2016 ACR/ARHP Annual Meeting

    Cell-Bound Complement Activation Products Correlate with Disease Activity in Childhood-Onset Systemic Lupus Erythematosus

    Joyce Hui-Yuen1, Derren Barken2, John Conklin3, Tyler O'Malley4, Andrew Eichenfield5, Amy Starr6, Lisa F. Imundo7, Thierry Dervieux8 and Anca D. Askanase9, 1North Shore-Long Island Jewish Health System, Lake Success, NY, 2Exagen Diagnostics, Inc., Vista, CA, 31261 Liberty Way Suite C, Exagen Diagnostics, Vista, CA, 4Research and Development, Exagen Diagnostics, Vista, CA, 5Morgan Stanley Children's Hospital of NY-Presbyterian, Columbia University, New York, NY, 6Pediatric Rheumatology, Columbia University Medical Center, New York, NY, 7Assoociate Professor of Pediatrics in Medicine - Rheumatology, Columbia University Medical Center, New York, NY, 8Research and Development, Exagen Diagnostics, Inc., Vista, CA, 9Department of Medicine, Rheumatology, Columbia University College of Physicians & Surgeons, New York, NY

    Background/Purpose: Elevated levels of cell-bound complement activation products (C4d deposition on erythrocytes [EC4d] and B lymphocytes [BC4d], CB-CAPs) have been demonstrated to be sensitive and…
  • Abstract Number: 2422 • 2016 ACR/ARHP Annual Meeting

    Cyclic Amp, Erk5, and Transdifferentiation of Cardiac Fibroblasts in the Pathogenesis of Autoimmune Congenital Heart Block

    Androo Markham1, Sara Rasmussen2, Miki Blumenberg3, Robert M Clancy2 and Jill P. Buyon1, 1Medicine, New York University School of Medicine, New York, NY, 2Department of Medicine, Division of Rheumatology, New York University School of Medicine, New York, NY, 3Dermatology, NYU School of Medicine, New York, NY

    Background/Purpose: Maternal autoantibodies (Ab) reactive with the Ro/La ribonucleoprotein complex are associated with the development of cardiac injury in a fetus passively exposed to these…
  • Abstract Number: 2423 • 2016 ACR/ARHP Annual Meeting

    Differential Interferon Score Expression in the Peripheral Blood in Mendelian Inflammatory Interferonopathies Versus Juvenile Dermatomyositis (JDM) Subtyped By Myositis Autoantibodies and Disease Activity

    Hanna Kim1, Terrance P. O'Hanlon2, Adriana Almeida de Jesus3, Yan Huang3, Ira N. Targoff4,5, Frederick W. Miller2, Raphaela Goldbach-Mansky6 and Lisa G. Rider2, 1NIAMS/NIH, Bethesda, MD, 2Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 3National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, 4Oklahoma Medical Research Foundation, Oklahoma City, OK, 5University of Oklahoma, Oklahoma City, OK, 6Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD

    Background/Purpose: JDM is a complex autoimmune disease with an interferon (IFN) signature, with a reported correlation with disease activity.  Clinical features vary among myositis-specific autoantibody…
  • Abstract Number: 2424 • 2016 ACR/ARHP Annual Meeting

    Novel Autoantigens for Anti- Endothelial Cell Antibodies in Pediatric Rheumatic Diseases Identified By Proteomics

    Rie Karasawa1, Mayumi Tamaki1, Toshiko Sato1, Kazuo Yudoh2 and James Jarvis3, 1Institute of Medical Science, St. Marianna University School of Medicine, Kawasaki, Japan, 2Institute of Medical Science, St. Marianna University School of Medicine, Kanagawa, Japan, 3Pediatrics, The University at Buffalo, Buffalo, NY

    Background/Purpose:  Juvenile dermatomyositis (JDM), a systemic autoimmune vasculopathy, and juvenile idiopathic arthritis (JIA) are representative rheumatic diseases in children. However the pathogenesis of these diseases…
  • Abstract Number: 2425 • 2016 ACR/ARHP Annual Meeting

    The Vasculopathy of Juvenile Dermatomyositis (JDM); Evidence of Persistent Endothelial Injury, Hypercoagulability, Subclinical Inflammation and Increased Arterial Stiffness

    Charalampia Papadopoulou1,2, Ying Hong1, Petra Krol1,2, Yiannis Ioannou3, Clarissa Pilkington2,4,5, Hema Chaplin6, Stephanie Simou1, Marietta Charakida7, Lucy R Wedderburn5,8,9, Paul Brogan10 and Despina Eleftheriou1,8,11, 1Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, 2Paediatric Rheumatology, Great Ormond Street Hospital NHS Trust, London, United Kingdom, 3Rayne Institute, Arthritis Research UK Centre for Adolescent Rheumatology, UCL Division of Medicine, London, United Kingdom, 4Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 5Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, United Kingdom, 6Centre for Adolescent Rheumatology, Arthritis Research UK, London, United Kingdom, 7Vascular Physiology Unit, Institute of Cardiovascular Science , University College London, London, United Kingdom, 8Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 9Rheumatology Unit, Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 10Department of Paediatric Rheumatology, UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 11Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom

    Background/Purpose:  Vasculopathy is considered central to the pathogenesis of Juvenile Dermatomyositis (JDM). The interplay between persistent JDM-vasculopathy, traditional cardiovascular risk factors, exposure to corticosteroids, and…
  • Abstract Number: 2426 • 2016 ACR/ARHP Annual Meeting

    Transcriptomic Analysis of Immune Subsets in Juvenile Dermatomyositis before and after Treatment Identifies Novel Pathways Involved in Pathogenesis

    Claire Deakin1, Georg Otto2,3, Meredyth Wilkinson4, Stefanie Dowle2,3, Stefania Simou5, Lucy Marshall6, Elizabeth Rosser6, Daniel Kelberman2,3, Lucy R Wedderburn6,7,8 and Juvenile Dermatomyositis Research Group (JDRG), 1Infection, Inflammation and Rheumatology Section,, UCL Institute of Child Health, London, United Kingdom, 2Genetics & Genomic Medicine Programme, UCL Institute of Child Health, London, United Kingdom, 3National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 4Division of Medicine, University College London, London, United Kingdom, 5Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, 6Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, United Kingdom, 7Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 8Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

    Background/Purpose:  Although proximal muscle weakness and skin rash are the typical features of juvenile dermatomyositis (JDM), little is known about disease pathogenesis, why other features…
  • Abstract Number: 2427 • 2016 ACR/ARHP Annual Meeting

    Identification of Biomarkers Using Tear Proteomics in Children with Chronic Anterior Uveitis

    Sheila Angeles-Han1,2, Duc Duong3, Steven Yeh4, Purnima Patel1, Kirsten Jenkins2, Sampath Prahalad2,5 and Gary Holland6, 1Emory University School of Medicine, Atlanta, GA, 2Children's Healthcare of Atlanta, Atlanta, GA, 3Emory University, Atlanta, GA, 4Ophthalmology, Emory University School of Medicine, Atlanta, GA, 5Pediatric Rheumatology, Emory University School of Medicine, Atlanta, GA, 6Jules Stein Eye Institute, Jules Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA

    Background/Purpose: Children with juvenile idiopathic arthritis (JIA) are at risk for anterior uveitis which can lead to ocular complications and vision loss.  The ANA is…
  • Abstract Number: 2428 • 2016 ACR/ARHP Annual Meeting

    Autoantibody Diversity in Pediatric Patients Undergoing Evaluation for Autoimmune Encephalitis: A Retrospective Investigation

    Anne E Tebo1, Thomas Haven2, Aimee O. Hersh3 and Eyal Muscal4, 1Pathology, University of Utah School of Medicine and ARUP Laboratories, Salt Lake City, UT, 2Department of Pathology, University of Utah, ARUP Institute of Clinical and Experimental Pathology, Salt Lake City, UT, 3Pediatrics/Rheumatology, University of Utah, Salt Lake City, UT, 4Immunology, allergy and Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX

    Background/Purpose:  Antibodies directed against N-methyl-D-aspartate type glutamate receptor (NMDAR), voltage-gated potassium channel (VGKC) or glutamic acid decarboxylase (GAD65) are frequently associated with autoimmune encephalitis (AE)…
  • Abstract Number: 2429 • 2016 ACR/ARHP Annual Meeting

    Mechanisms for the Development of Lung Fibrosis in Sting-Associated Vasculopathy with Onset in Infancy (SAVI)

    Adriana Almeida de Jesus1, Louise Malle1, Dan Yang2, Bernadette Marrero1, Yin Liu3, Gina A. Montealegre Sanchez1, Dawn C. Chapelle4, Hanna Kim4, Michelle O'Brien4, Gregor Dueckers5, Suzanne Ramsey6, Joseph R. Fontana7, Steven M. Holland8, Yan Huang1, Suvimol Hill9, Laisa Santiago10, Benito Gonzalez11, Paul Brogan12, Juergen Brunner13, Ebun Omoyinmi14, Athimalaipet V Ramanan15, Amy Paller16, Olcay Y. Jones17, Seza Ozen18, Stephen Brooks4, Zuoming Deng4, Manfred Boehm19, Raphaela Goldbach-Mansky20 and Helmut Wittkowski21, 1National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, 2National Heart, Lung, and Blood Institute (NHLBI), NIH, Bethesda, MD, 3Scientific Review Branch, NIAMS, NIH, Bethesda, MD, 4NIAMS/NIH, Bethesda, MD, 5Helios Kliniken - Kinderklinik, HELIOS Klinikum Krefeld, Krefeld, Germany, 6Pediatric Rheumatology, IWK Health Centre, Halifax, NS, Canada, 7Cardiovascular and Pulmonary Branch, NHLBI, NIH, Bethesda, MD, 8Laboratory of Clinical Infectious Disease, NIAID, NIH, Bethesda, MD, 9Radiology Department, Clinical Center, NIH, Bethesda, MD, 10Johns Hopkins All Children's Hospital Rheumatology, Saint Petersburg, FL, 11Luis Calvo Mackenna Hospital, Santiago, Chile, 12UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 13Department of Pediatrics, Medical University Innsbruck, Innsbruck, Austria, 14University College London Institute of Child Health, London, United Kingdom, 15University Hospitals Bristol NHS Foundation Trust, Bristol, United Kingdom, 16Departments of Dermatology and Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA;, Chicago, IL, 17Pediatrics, Walter Reed National Military Medical Center, Bethesda, MD, 18Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, ANKARA, Turkey, 19Laboratory of Cardiovascular Regenerative Medicine, National Heart, Lung, and Blood Institute (NHLBI), NIH, Bethesda, MD, 20Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, 21Department of Pediatric Rheumatology and Immunology, University Hospital of Muenster, Münster, Germany

    Background/Purpose:  STING-Associated Vasculopathy with Onset in Infancy (SAVI) is a monogenic autoinflammatory interferonopathy caused by gain-of-function mutations in TMEM173/STING, a nucleic acid sensor adaptor linked…
  • Abstract Number: 2430 • 2016 ACR/ARHP Annual Meeting

    Mutations in the Tyrosine-Protein Kinase Lyn Cause an Early-Onset Neutrophilic Vasculitis Syndrome

    Adriana Almeida de Jesus1, Gina A. Montealegre1, Helen Freeman2, Neil Martin3, Ebun Omoyinmi4, Bernadette Marrero1, Katherine R. Calvo5, Chyi-Chia Richard Lee6, April D. Brundidge7, David Kleiner8, Stephen Hewitt8, Dawn C. Chapelle7, Yan Huang1, Nirali Shah8, Stephen Brooks7, Eric Meffre9, Paul Brogan10, Hyesun Kuehn11, Sergio Rosenzweig12, Melinda Merchant8, Zuoming Deng7, Susan Moir13 and Raphaela Goldbach-Mansky14, 1National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, 2Raigmore Hospital, Inverness, United Kingdom, 3Royal Hospital for Children, Glasgow, United Kingdom, 4University College London Institute of Child Health, London, United Kingdom, 5Department of Laboratory Medicine, Hematology Section, National Institutes of Health Clinical Center, Bethesda, MD, 6Dermatopathology Section, Laboratory of Pathology, National Cancer Institute (NCI), National Institutes of Health (NIH), Bethesda, MD, 7NIAMS/NIH, Bethesda, MD, 8National Cancer Institute, NIH, Bethesda, MD, 9Department of Immunobiology, Yale University School of Medicine, New Haven, CT, 10UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 11Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD, 12Department of Laboratory Medicine/NIH, Bethesda, MD, 13National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 14Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD

    Background/Purpose: Tyrosine-protein kinase Lyn is a Src-family tyrosine kinase expressed by hematopoietic and non-hematopoietic cell types. Phosphorylation of a tyrosine residue at position 508 renders…
  • Abstract Number: 2431 • 2016 ACR/ARHP Annual Meeting

    Characterization of Innate Immune Cells in Patients with the Interferon-Mediated Autoinflammatory Diseases Sting Associated Vasculopathy with Onset in Infancy (SAVI) and Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE)

    Bernadette Marrero1, Yin Liu2, Katherine R. Calvo3, Angelique Biancotto4, Yan Huang1, Adriana Almeida de Jesus1, Gina A. Montealegre Sanchez1 and Raphaela Goldbach-Mansky5, 1National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, 2Scientific Review Branch, NIAMS, NIH, Bethesda, MD, 3Department of Laboratory Medicine, Hematology Section, National Institutes of Health Clinical Center, Bethesda, MD, 4Center for Human Immunology, Autoimmunity and Inflammation, NHLBI/NIH, Bethesda, MD, 5Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD

    Background/Purpose:   We recently described two rare autoinflammatory interferonopathies, STING Associated Vasculopathy with Onset in Infancy (SAVI) and Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and…
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