Abstracts Archive - Page 1543 of 2607 - ACR Meeting Abstracts

Abstract Number: 2336 • 2017 ACR/ARHP Annual Meeting
Expression of Myxovirus-Resistance Protein a: A Possible Marker of Muscular Disease Activity in Juvenile Dermatomyositis
Sirisucha Soponkanaporn¹, Claire Deakin², Lucy Marshall², Cerise Johnson², Peter Schutz¹ and Lucy R Wedderburn², ¹Infection Inflammation and Rheumatology Section, UCL Great Ormond Street Institute of Child Health, University College London, United Kingdom, London, United Kingdom, ²Infection, Immunity and Inflammation Programme, UCL Great Ormond Street Institute of Child Health, University College London, United Kingdom, London, United Kingdom
Background/Purpose: Juvenile dermatomyositis (JDM) is a chronic autoimmune myopathy characterized by proximal muscle weakness and typical skin rashes. Type I interferon (IFN) gene expression in…
Abstract Number: 2337 • 2017 ACR/ARHP Annual Meeting
Novel Serum Broad-Based Proteomic Discovery Analysis Identifies Proteins and Pathways Dysregulated in Juvenile Dermatomyositis (JDM) and Myositis Autoantibody Groups
Hanna Kim¹, Angélique Biancotto², Foo Cheung³, Terrance P. O'Hanlon⁴, Ira N. Targoff⁵, Yan Huang⁶, Frederick W Miller⁴, Raphaela Goldbach-Mansky⁶ and Lisa G Rider⁴, ¹Pediatric Translational Research Branch, NIAMS/NIH, Bethesda, MD, ²Center for Human Immunology, Autoimmunity and Inflammation (CHI), NHLBI, NIH, Bethesda, MD, ³Center for Human Immunology Autoimmunity and Inflammation (CHI), NHLBI, NIH, Bethesda, MD, ⁴Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, ⁵VA Medical Center, University of Oklahoma Health Sciences Center, Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁶Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD
Background/Purpose: Juvenile dermatomyositis (JDM) is a complex heterogeneous autoimmune disease. Myositis-specific autoantibodies (MSAs), present in up to 80% of JDM patients, help define distinct phenotypes…
Abstract Number: 2338 • 2017 ACR/ARHP Annual Meeting
Plasma Exosomes from Children with Juvenile Dermatomyositis Are Taken up By Human Aortic Endothelial Cells and Are Associated with Altered Gene Expression in Those Cells
Kaiyu Jiang¹, Zihua Hu², Rie Karasawa³, Yanmin Chen¹ and James Jarvis⁴, ¹Pediatrics, University at Buffalo, Buffalo, NY, ²Center for Computational Research, University at Buffalo, Buffalo, NY, ³Institute of Medical Science, St. Marianna University School of Medicine, Japan, Kawasaki, Japan, ⁴Department of Genetics, Genomics & Bioinformatics, University at Buffalo, Buffalo, NY
Background/Purpose: The pathology of juvenile dermatomyositis (JDM) is characterized by prominent vessel wall and perivascular inflammation. This feature of the disease has remained unexplained and…
Abstract Number: 2339 • 2017 ACR/ARHP Annual Meeting
Expression of Type I and Type II Interferons Is Increased in Muscle Biopsies of Juvenile Dermatomyositis Patients and Related to Clinical and Histological Features
Rebecca Nicolai¹, Gian Marco Moneta², Silvia Rosina³, Chiara Fiorillo³, Denise Pires Marafon¹, Margherita Verardo⁴, Luisa Bracci Laudiero^5,6, Carlo Minetti^3,7, Angelo Ravelli^3,8 and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Bambino Gesù Children's Hospital, Rome, Rome, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Roma, Italy, ³University of Genova, Genova, Italy, ⁴Unit of Neuromuscular and Neurodegenerative Disorders, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Roma, Italy, ⁵Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Roma, Italy, ⁶Institute of Translational Pharmacology, CNR, Rome, Italy, Rome, Italy, ⁷Pediatric Neurology and Muscular Disorders, Giannina Gaslini Institute, Genova, Italy, ⁸Rheumatology, Giannina Gaslini Institute, Genova, Italy
Background/Purpose: There is growing evidence for an involvement of interferons (IFNs) in the chronic inflammation that characterizes juvenile dermatomyositis (JDM). The aim of this study…
Abstract Number: 2340 • 2017 ACR/ARHP Annual Meeting
Correlation of Type I Interferon Score and CXCL10 (C-X-C Motif Chemokine Ligand 10) with Cutaneous and Muscular Disease Activity in Juvenile Dermatomyositis Patients
Rebecca Nicolai¹, Gian Marco Moneta², Ivan Caiello³, Denise Pires Marafon¹, Silvia Rosina⁴, Luisa Bracci Laudiero^5,6, Angelo Ravelli^4,7 and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Bambino Gesù Children's Hospital, Rome, Rome, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Roma, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ⁴University of Genova, Genova, Italy, ⁵Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Roma, Italy, ⁶Institute of Translational Pharmacology, CNR, Rome, Italy, Rome, Italy, ⁷Rheumatology, Giannina Gaslini Institute, Genova, Italy
Background/Purpose: Interferons (IFNs) seem to be important contributors in the pathogenesis of juvenile dermatomyositis (JDM). Our group previously reported that expression of both type I…
Abstract Number: 2341 • 2017 ACR/ARHP Annual Meeting
The Interaction between Genetic Risk Factors and Age of Disease Onset in Juvenile Dermatomyositis
Claire Deakin¹, John Bowes², Lucy Marshall¹, Cerise Johnson¹, Gulnara Mamyrova³, Rodolfo Curiel⁴, Kelly A. Rouster-Stevens⁵, Heinrike Schmeling⁶, Adam Huber⁷, Brian M. Feldman⁸, Ann M Reed⁹, Lauren M. Pachman¹⁰, Soumya Raychaudhuri¹¹, Stephen Eyre¹² and Lucy R Wedderburn¹, ¹Infection, Immunity and Inflammation Programme, UCL Great Ormond Street Institute of Child Health, University College London, United Kingdom, London, United Kingdom, ²Arthritis Research UK Epidemiology Unit, The University of Manchester, Manchester, United Kingdom, ³Rheumatology, George Washington University School of Medicine and Health Sciences, Washington, DC, ⁴Department of Rheumatology, George Washington University, Washington, DC, ⁵Pediatric Rheumatology, Emory Children's Center, Atlanta, GA, ⁶Alberta Children’s Hospital/University of Calgary, Calgary, AB, Canada, ⁷IWK Health Centre, Halifax, NS, Canada, ⁸Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ⁹Rheumatology, Duke University, Durham, NC, ¹⁰Cure 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, ¹¹Division of Medicine and Rheumatology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, ¹²Arthritis Research UK Centre for Genetics and Genomics, Centre for Musculoskeletal Research, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, The University of Manchester, Manchester, United Kingdom
Background/Purpose: Juvenile dermatomyositis (JDM) is a rare, severe autoimmune disease characterized by muscle weakness and rash. Clinical features of JDM are heterogeneous, and can include…
Abstract Number: 2342 • 2017 ACR/ARHP Annual Meeting
Transcriptomic Analysis Reveals Mitochondrial and Monocyte Dysfunctions Are Linked to the Interferonopathy of Juvenile Dermatomyositis
Claire Deakin¹, Elizabeth Rosser¹, Lucy Marshall¹, Meredyth Wilkinson², Aziza Khabbush³, Stefania Simou¹, Georg Otto³, Stefanie Dowle³, Daniel Kelberman³, Simon Yona², Simon Eaton³ and Lucy R Wedderburn¹, ¹Infection, Immunity and Inflammation Programme, UCL Great Ormond Street Institute of Child Health, University College London, United Kingdom, London, United Kingdom, ²Division of Medicine, University College London, London, United Kingdom, ³Genetics and Genomic Medicine Programme, UCL Great Ormond Street Institute of Child Health, University College London, United Kingdom, London, United Kingdom
Background/Purpose: Although type I interferon (IFN1) and endoplasmic reticulum (ER) stress have been implicated in pathogenesis of juvenile dermatomyositis (JDM), little else is known about…
Abstract Number: 2343 • 2017 ACR/ARHP Annual Meeting
A Genome-Wide Association Study Suggests the HLA Class II Region As the Major Susceptibility Locus for IgA Vasculitis
Raquel López-Mejías¹, Sara Remuzgo-Martínez¹, Fernanda Genre¹, Francisco David Carmona², Santos Castañeda³, Belén Sevilla Pérez⁴, Norberto Ortego Centeno⁴, Javier Llorca⁵, Begoña Ubilla¹, Veronica Mijares¹, Trinitario Pina⁶, Jose A. Miranda-Filloy⁷, Antonio Navas Parejo⁸, Diego Argila⁹, Maximiliano Aragües¹⁰, Esteban Rubio-Romero¹¹, Manuel Leon Luque¹², Juan María Blanco Madrigal¹³, Eva Galindez-Agirregoikoa¹³, David Jayne¹⁴, Ricardo Blanco¹, Javier Martin¹⁵ and Miguel Angel González-Gay¹⁶, ¹Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain, ²Instituto de Parasitología y Biomedicina López-Neyra, IPBLN-CSIC, PTS-Granada, Departamento de Genética e Instituto de Biotecnología, University of Granada, Granada, Spain, ³Hospital Universitario La Princesa. IIS-IP. Madrid. Spain, Madrid, Spain, ⁴Medicine Department, Hospital Universitario San Cecilio, Granada, Spain, ⁵Department of Epidemiology and Computational Biology, School of Medicine, University of Cantabria, and CIBER Epidemiología y Salud Pública (CIBERESP), IDIVAL, Santander, Spain, ⁶Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, IDIVAL, Santander, Spain, ⁷Rheumatology Division, Hospital Lucus Augusti, Lugo, Spain, ⁸Nephrology Department, Hospital Universitario San Cecilio, Granada, Spain, ⁹Dermatology Department, Hospital Universitario La Princesa, IIS-Princesa, Madrid, Spain, ¹⁰Dermatology Department, Hospital Universitario La Princesa, IIS-Princesa, MAdrid, Spain, ¹¹Hospital Universitario Virgen del Rocío. Sevilla. Spain, Sevilla, Spain, ¹²Rheumatology Department,Hospital Universitario Virgen del Rocío, Sevilla, Spain, ¹³Rheumatology Division, Hospital Universitario de Basurto, Bilbao, Spain, ¹⁴Department of Medicine, University of Cambridge, Cambridge, United Kingdom, ¹⁵Instituto de Parasitología y Biomedicina López-Neyra, Granada, Spain, ¹⁶Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, IDIVAL and School of Medicine, University of Cantabria, Santander, Spain
Background/Purpose: Immunoglobulin-A (IgA) vasculitis, also known as Henoch-Schöenlein purpura (HSP), is the most common type of primary small-sized blood vessel leukocytoclastic vasculitis in children, although…
Abstract Number: 2344 • 2017 ACR/ARHP Annual Meeting
Cell-Bound Complement Activation Products As Markers of Disease Flares in Childhood Onset Systemic Lupus Erythematosus
Yevgeniya Gartshteyn¹, Joyce Hui-Yuen², Miya Okado¹, Teja Kapoor¹, Thierry Dervieux³ and Anca Askanase⁴, ¹Rheumatology, Columbia University College of Physicians & Surgeons, New York, NY, ²Pediatric Rheumatology, Columbia University College of Physicians & Surgeons, New York, NY, ³3Exagen Diagnostics, Albuquerque, CA, ⁴Department of Medicine, Rheumatology, Columbia University College of Physicians & Surgeons, New York, NY
Background/Purpose: Cell-bound complement activation products, CB-CAPs (C4d deposition on erythrocytes [EC4d], B lymphocytes [BC4d], reticulocytes [RC4d], platelets [PC4d], and C3d deposition on reticulocytes [RC3d]) are…
Abstract Number: 2345 • 2017 ACR/ARHP Annual Meeting
Characterization of Adenosine Deaminase 2 Variants Identified in an International Pediatric Vasculitis Cohort
Kristen Gibson^1,2, David Cabral^3,4, Britt Drogemoller⁵, Xiaohua Xhan⁵, Fudan Miao⁶, Kimberly Morishita⁷, Erin Gill⁸, Robert E. W. Hancock⁸, Colin Ross^5,9 and Kelly Brown^9,10, ¹Medical Genetics, The University of British Columbia, Vancouver, BC, Canada, ²BC Children's Hospital Research Insitute, Vancouver, BC, Canada, ³BC Children's Hospital, Vancouver, BC, Canada, ⁴University of British Columbia, Vancouver, BC, Canada, ⁵Pharmaceutical Sciences, The University of British Columbia, Vancouver, BC, Canada, ⁶The University of British Columbia, Vancouver, BC, Canada, ⁷BC Children's Hospital and University of British Columbia, Vancouver, BC, Canada, ⁸Microbiology and Immunology, The University of British Columbia, Vancouver, BC, Canada, ⁹BC Children's Hospital Research Institute, Vancouver, BC, Canada, ¹⁰Pediatrics, The University of British Columbia, Vancouver, BC, Canada
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a recently recognized, autosomal recessive genetic disease. Patients present with various, early-onset systemic vascular and inflammatory manifestations,…
Abstract Number: 2346 • 2017 ACR/ARHP Annual Meeting
Chemokine Ligand 9 (CXCL9) [Monokine Induced By Gamma Interferon (MIG)] As a Predictor of Active Disease Status in Localized Scleroderma
Kathryn S. Torok¹, Qi Mi², Emily Mirizio³, Kaila Schollaert-Fitch¹, Mark Fritzler⁴ and Marvin J. Fritzler⁵, ¹Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, ²Department of Sports Medicine and Nutrition, University of Pittsburgh, Pittsburgh, PA, ³Pediatric Rhuematology, University of Pittsburgh Med Ctr, Pittsburgh, PA, ⁴Eve Technologies, Calgary, AB, Canada, ⁵Medicine, University of Calgary, Calgary, AB, Canada
Background/Purpose: Localized scleroderma (LS) is a fibrotic autoimmune disease of the skin and underlying tissues which can lead to disfiguring sequlea, especially in childhood-onset. Untreated…
Abstract Number: 2347 • 2017 ACR/ARHP Annual Meeting
High Interferon (IFN) Signatures and Overlapping Clinical Features Characterize Subgroups of Patients with Presumed IFN-Mediated Autoinflammatory Diseases
Adriana Almeida de Jesus¹, Yanfeng Hou², Louise Malle¹, Scott Canna³, Stephen R. Brooks⁴, Hanna Kim⁵, Gina A. Montealegre Sanchez¹, Rachel VanTries¹, Angélique Biancotto⁶, Samantha Dill⁵, Dawn C. Chapelle⁵, Bernadette Marrero¹, Yan Huang¹ and Raphaela Goldbach-Mansky¹, ¹Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, ²Department of Rheumatology, Shandong Provincial Qianfoshan Hospital, Shandong University, Shandong, China, ³Richard King Mellon Foundation Institute for Pediatric Research, Children's Hospital of Pittsburgh, Pittsburrgh, PA, ⁴Biodata Mining and Discovery Section, Office of Science and Technology, NIAMS/NIH, Bethesda, MD, ⁵Pediatric Translational Research Branch, NIAMS/NIH, Bethesda, MD, ⁶Center for Human Immunology, Autoimmunity and Inflammation (CHI), NHLBI, NIH, Bethesda, MD
Background/Purpose: Many pediatric patients (pts.) with early-onset autoinflammatory disease (AID) phenotypes are mutation-negative for genetically known AIDs. Recent data suggest a role for Type-I interferon…
Abstract Number: 2348 • 2017 ACR/ARHP Annual Meeting
A New Cause of Mendelian Lupus Due to IKZF1 Mutation Underlines the B Cell Landscape Heterogeneity in Monogenic Lupus
Alexandre Belot^1,2, Cecile Frachette³, Omarjee Sulliman Ommar², Anne-Laure Mathieu², Thibault Andrieu², Paul Mondier², Gillian Rice⁴, Heloise Reumaux⁵, David Launay^6,7, Marc Lambert^8,9, Guillaume Lefevre¹⁰, Nicole Fabien¹¹, Christophe Malcus¹², Isabelle Rouvet¹³, Emilie Chopin¹³, Anne-Sophie Michallet¹⁴, Thierry Defrance¹⁴, Thierry Walzer¹⁴ and Yanick J Crow¹⁵, ¹Pediatric Rheumatology Unit, HFME, Hospices Civils de Lyon, Bron, France, ²U1111, INSERM, Lyon, France, ³Hospices Civils de Lyon, Bron, France, ⁴Manchester Academic Health Science Centre, Institute of Human Development, University of Manchester, Manchester, United Kingdom, ⁵Pediatric Rheumatology, CHRU Lille, Lille, France, ⁶LIRIC, INSERM UMR 995, Lille, France, ⁷Service de Médecine Interne, Centre National de Référence des Maladies Systémiques Rares, Hôpital Claude Huriez, CHRU Lille, Lille, France, ⁸Univ. Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France, Lille, France, ⁹Medecine Interne Lilloise, Service de médecine interne, Hôpital Claude Huriez, Université Lille II, Lille, France, Lille, France, ¹⁰Internal Medicine, Lille University Hospital, Lille, France, ¹¹Immunology Department, UF d'Autoimmunité, Immunology Department, CHU de Lyon HCL- GH Sud, Lyon, France, ¹²Cellular Immunology Department - Laboratoire d'immunologie cellulaire, Groupement Hospitalier Edouard Herriot, Lyon, France, ¹³Cellular Biotechnology Department, Centre de biologie et pathologie Est, Hospices civils de Lyon, Lyon, France, ¹⁴International Center for Infectiology Research - CIRI, Lyon, France, ¹⁵Institut Imagine, Paris, France
Background/Purpose: Next generation sequencing (NGS) represents a revolution in the field of molecular medicine, and offers a new approach to deciphering the pathogenesis of complex…
Abstract Number: 2349 • 2017 ACR/ARHP Annual Meeting
Interferon Signature in Childhood Rheumatic Diseases
Hafize Emine Sonmez¹, İ. Çağatay Karaaslan², Ezgi Deniz Batu³, Banu Anlar⁴, Betul Sozeri⁵, Adriana Almeida de Jesus⁶, Raphaela Goldbach-Mansky⁷ and Seza Ozen⁸, ¹Department of Pediatrics, Divison of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ²Department of Molecular Biology, Hacettepe University, Ankara, Turkey, ³Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁴Department of Pediatrics, Division of Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁵Pediatric Rheumatology, Ümraniye Tranning and Research Hospital, İstanbul, Turkey, ⁶National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, ⁷Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, ⁸Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Background/Purpose: Several rheumatic diseases are characterized by overexpression of type I interferon(IFN)-inducible or viral response genes, termed the IFN signature. Recently this signature has been…
Abstract Number: 2350 • 2017 ACR/ARHP Annual Meeting
Gastrointestinal Microbiota in New-Onset Juvenile Idiopathic Arthritis
Sriharsha Grevich^1,2, Kyle Hager³, Mitchell Brittnacher³, Hillary Hayden³, Sarah Ringold^1,2, David Suskind^1,2, Samuel Miller³ and Anne Stevens^1,2, ¹University of Washington, Department of Pediatrics, Seattle, WA, ²Seattle Children's Hospital, Seattle, WA, ³University of Washington, Department of Microbiology, Seattle, WA
Background/Purpose: Oral and gut microbes have been implicated in the pathogenesis of rheumatoid arthritis, spondyloarthropathy, and juvenile enthesitis-related arthritis (ERA). Pro-inflammatory microbes in the bacteroides…

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