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  • Abstract Number: 3109 • 2016 ACR/ARHP Annual Meeting

    Regulatory T Cells Deficient in Protein Phosphatase 2A Lose Suppressive Function and Convert to an Effector Phenotype

    Isaac R. Kasper1, Hao Li1, Sokratis Apostolidis2, Maria G. Tsokos3 and George C. Tsokos4, 1Rheumatology, Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, MA, 2Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, MA, 3Beth Israel Deaconess Medical Center, Boston, MA, 4Medicine/Rheumatology, Harvard Medical School Beth Israel, Boston, MA

    Background/Purpose: Regulatory T cells (Tregs) represent the fundamental T-cell capable of promoting self-tolerance and balancing excessive inflammation. Quantitative and qualitative Treg deficiencies have been characterized…
  • Abstract Number: 3110 • 2016 ACR/ARHP Annual Meeting

    Rab4A Is Required for Development of Tregs, Restricts Antiphospholipid Antibody Production and Pro-Inflammatory Expansion of Macrophages and Neutrophils, and Blocks Pristane-Induced Intra-Alveolar Hemorrhage in a Mouse Model of SLE

    Zachary Oaks1, Thomas Winans1, Nick Huang1, Sarah Blair1, Miguel Beckford1, Katalin Banki2 and Andras Perl3, 1SUNY, Syracuse, NY, 2Clinical Pathology, SUNY Upstate Medical University, Syracuse, NY, 3Department of Medicine, Upstate Medical University, Syracuse, NY

    Background/Purpose: Polymorphic haplotypes of the HRES-1 endogenous retrovirus at the 1q42 chromosomal locus have been associated with lupus susceptibility, in particular, with predisposition to antiphospholipid…
  • Abstract Number: 3111 • 2016 ACR/ARHP Annual Meeting

    Pathogenic T Cell Responses in Systemic Sclerosis Is Shaped By Novel Cytokine Axis in the Microenvironment: A Multidimensional, High Throughput Analysis

    Hari Balaji1, Andrea HL Low2, Chieh Hwee Ang3, Raymond Ong Jr.4, Juntao Li5, Camillus Chua3, Liyun Lai3, Suzan Saidin3 and Salvatore Albani3, 1SingHealth Translational Immunology and Inflammation Centre, Singapore, Singapore, 2-, Singapore, Singapore, 3SingHealth Translational Immunology and Inflammation Centre, Singapore Health Services Pte Ltd, Singapore, Singapore, 4Singhealth Translational Immunology and Inflammation Centre (STIIC), Singapore Health Services Pte Ltd, Singapore, Singapore, 5Duke-National University of Singapore Graduate Medical School, Singapore, Singapore

    Background/Purpose: Pathogenic immune responses can be profoundly shaped by the interplay between the periphery and the microenvironment. In this work we aimed at defining the…
  • Abstract Number: 3112 • 2016 ACR/ARHP Annual Meeting

    DOCK8 Associates with STAT5 and Promotes Regulatory T Cell Function

    Erin Janssen1, Mira Tohme1, Sumana Ullas2 and Raif Geha2, 1Immunology, Boston Children's Hospital, Boston, MA, 2Boston Children's Hospital, Boston, MA

    Background/Purpose: DOCK8 deficiency leads to autosomal recessive Hyper-IgE syndrome (AR-HIES). DOCK8 deficient patients develop recurrent sinopulmonary infections, chronic mucocutaneous viral infections, hyper-IgE, eosinophilia, food allergies,…
  • Abstract Number: 3113 • 2016 ACR/ARHP Annual Meeting

    Evaluation of a Clinical Transition Pathway for Adolescents with Autoimmune Diseases

    Margot Walter1, Sylvia S.M. Kamphuis2,3, Philomine A. van Pelt4, A. Vroed de5 and Johanna M.W. Hazes6, 1Rheumatology, Erasmus MC - University Medical Center, Rotterdam, Netherlands, 2Erasmus Medical Center, Rotterdam, Netherlands, 3Pediatric Rheumatology, Sophia Children's Hospital – Erasmus University Medical Centre, Rotterdam, Netherlands, 4Rheumatology, Erasmus MC, Rotterdam, Netherlands, 5ErasmusMC, Rotterdam, Netherlands, 6Department of Rheumatology, Erasmus University Medical Centre, Rotterdam, Netherlands

    Background/Purpose: It is clear that young people (YP) with an autoimmune disease need a transitional process before they are transferred to the adult clinic. Therefore,…
  • Abstract Number: 3114 • 2016 ACR/ARHP Annual Meeting

    Cardiorespiratory Fitness in Children with Juvenile Idiopathic Arthritis Treated in the Biological Era Is Comparable with Controls- a Cross-Sectional Study

    Kristine Risum1, Elisabeth Edvardsen2,3, Anne Marit Selvaag4, Oyvind Molberg4, Hanne Dagfinrud5 and Helga Sanner4,6, 1Department of Rehabilitation, Division of Orthopeadic Surgery, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 2Department of Sports Medicine, Norwegian School of Sport Sciences, Oslo, Norway, Oslo, Norway, 3Department of Pulmonary Medicine, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 4Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 5Department of Rheumatology, Diakonhjemmet Hospital, Oslo, Norway, Oslo, Norway, 6Norwegian National Advisory Unit on Rheumatic Diseases in Children and Adolescents, Oslo University Hospital, Oslo, Norway, Oslo, Norway

    Cardiorespiratory Fitness in Children with Juvenile Idiopathic Arthritis Treated in the Biological Era is Comparable with Controls- a Cross-Sectional Study    Background/Purpose: Reduced cardiorespiratory fitness (CRF)…
  • Abstract Number: 3115 • 2016 ACR/ARHP Annual Meeting

    Reconsidering the Juvenile Idiopathic Arthritis Core Set: How Patients and Caregivers Define Disease Activity

    Jennifer R. Horonjeff1, Susan Thornhill2, Daniel B. Horton3, Jennifer N. Stinson4, Anjali Fortna5, Stephanie Luca6, Arlene Vinci7, Laura C. Marrow8, Emily L. Creek7, Meredith Riebschleger9, Alessandro Consolaro10, Jane Munro11, Vibeke Strand12, Clifton Bingham III13 and Esi Morgan14, 1Rheumatology, Columbia University Medical Center, New York, NY, 2Thornhill Associates, Hermosa Beach, CA, 3Pediatrics, Division of Pediatric Rheumatology, Rutgers Robert Wood Johnson Medical School, Rutgers Biomedical and Health Sciences, New Brunswick, NJ, 4Anesthesia and Pain Medicine, The Hospital for Sick Children, Toronto, ON, Canada, 5Columbia University Medical Center, New York, NY, 6The Hospital for Sick Children, Toronto, ON, Canada, 7Consumer Health, Arthritis Foundation, Atlanta, GA, 8Arthritis Foundation, Atlanta, GA, 9Pediatric Rheumatology & Health Services Research, University of Michigan, Ann Arbor, MI, 10Pediatria II - Reumatologia, PRINTO, Istituto Giannina Gaslini, Genoa, Italy, 11Rheumatology, Royal Children's Hospital, Parkville, Australia, 12Stanford University School of Medicine, Palo Alto, CA, 13Divisions of Rheumatology and Allergy, Department of Medicine, Johns Hopkins University, Johns Hopkins University, Baltimore, MD, 14Pediatric Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH

    Background/Purpose: The current JIA Core Set (ACR Pediatric 30) contains items that should be assessed in clinical trials for children with JIA. It was developed…
  • Abstract Number: 3116 • 2016 ACR/ARHP Annual Meeting

    Risk of Infections in Juvenile Idiopathic Arthritis Patients Treated with Biologic Agentsand/or Methotrexate: Results from Pharmachild Registry

    Gabriella Giancane1, Joost Swart2, Francesca Bovis2, Elio Castagnola2, Andreas Groll2, Gerd Horneff2, Hans-Iko Huppertz2, Daniel J. Lovell2, Tom Wolfs2, Michaël Hofer2, Ekaterina Alexeeva2, Violeta Vladislava Panaviene2, Susan Nielsen2, Jordi Anton2, Florence Uettwiller2, Valda Stanevicha2, Maria Trachana2, Denise Pires Marafon3, Constantin Ailioaie2, Elena Tsitsami2, Sylvia S.M. Kamphuis2, Troels Herlin2, Pavla Dolezalová3, Gordana Susic2, Berit Flato2, Flavio Sztajnbok2, Angela Pistorio1, Alberto Martini2, Nico Wulffraat2 and Nicolino Ruperto2, 1Pediatria II, Reumatologia, PRINTO, Istituto Giannina Gaslini, Genoa, Italy, 2Istituto Giannina Gaslini, Genoa, Italy, 3Istituto Giannina Gaslini, genoa, Italy

    Background/Purpose: Pharmachild is an international registry involving over 100 the Paediatric Rheumatology INternational Trials Organisation (PRINTO)/ the Paediatric Rheumatology European Society (PRES) centres in 38 countries. The registry was set up to evaluate long term safety and efficacy…
  • Abstract Number: 3117 • 2016 ACR/ARHP Annual Meeting

    Development of Autoimmune Diseases and Genetic Predisposition in Children with Neonatal Lupus and Their Unaffected Siblings

    Aaron Garza Romero1, Peter M. Izmirly2, Hannah C. Ainsworth3, Miranda Marion3, Carl Langefeld3, Robert Clancy4, Jill P. Buyon5 and Amit Saxena6, 1Rheumatology, NYU School of Medicine, New York, NY, 2New York University School of Medicine, New York, NY, 3Wake Forest School of Medicine, Winston-Salem, NC, 4Department of Medicine, Division of Rheumatology, New York University School of Medicine, New York, NY, 5Medicine, New York University School of Medicine, New York, NY, 6Rheumatology, New York University School of Medicine, New York, NY

    Background/Purpose: Neonatal Lupus (NL) is a model of passively acquired autoimmunity conferred by exposure to maternal anti-Ro antibodies.  This study was initiated to address the…
  • Abstract Number: 3118 • 2016 ACR/ARHP Annual Meeting

    Differences in Disease Phenotype, Management and Outcomes of Children with Juvenile Idiopathic Arthritis throughout the World  – Analysis of 8,325 Patients Enrolled in the Epoca Study

    Alessandro Consolaro1,2, Nicolino Ruperto3, Pieter van Dijkhuizen4, Marco Garrone5, Mariangela Rinaldi5, Jaime De Inocencio6, Erkan Demirkaya7, Stella Maris Garay8, Dirk Föll9, Daniel J Lovell10, Calin Lazar11, Susan Nielsen12, Berit Flatø13, Alberto Martini1,2, Angelo Ravelli1,2 and Pediatric Rheumatology International Trials Organization and EPOCA Study Group, 1Istituto Giannina Gaslini, Genoa, Italy, 2University of Genova, Genova, Italy, 3Paediatric Rheumatology International Trials Organization (PRINTO), Istituto Giannina Gaslini, Genoa, Italy, 4Istituto Giannina Gaslini, Genova, Italy, 5PRINTO - Istituto Giannina Gaslini, Genova, Italy, 6Hospital 12 de Octubre, Madrid, Spain, 7Gulhane Military Medical Faculty, Ankara, Turkey, 8Hospital de Ninos, La Plata, Argentina, 9University Hospital Muenster, Muenster, Germany, 10PRCSG Cincinnati Children's Hospital Medical Center, Cinncinnati, OH, 11Clinica Pediatrie I, Cluj-Napoca, Romania, 12Rigshospitalet, Copenhagen, Denmark, 13Department of Rheumatology, Oslo University Hospital-Rikshospitalet, Oslo, Norway

    Background/Purpose: Several epidemiologic surveys have documented a remarkable, yet unexplained, disparity in the prevalence of juvenile idiopathic arthritis (JIA) subtypes among different geographic areas or…
  • Abstract Number: 3119 • 2016 ACR/ARHP Annual Meeting

    Complete Whole Genome Transcriptome, DNA Methylation, and Histone Mark Analysis of Rheumatoid Arthritis (RA) Fibroblast-like Synoviocytes (FLS) Reveals a Distinctive Epigenetic Landscape and Critical Pathogenic Pathways

    Rizi Ai1, Deepa Hammaker2, David L. Boyle3, Andre ‎ Wildberg4, Keisuke Maeshima2, Emmanuele Palescandolo5, Vinod Krishna5, Bryan Linggi6, Radu Dobrin5, John W. Whitaker7, Wei Wang8 and Gary Firestein9, 1Chemistry and Biochemistry, UC San Diego, La Jolla, CA, 2Division of Rheumatology, Allergy and Immunology, UCSD School of Medicine, La Jolla, CA, 3Division of Rheumatology, Allergy and Immunology, University of California, San Diego, La Jolla, CA, 4Chemistry and Biochemistry, UNIVERSITY OF CALIFORNIA SAN DIEGO, LA JOLLA, CA, 5Janssen Pharmaceuticals, Spring House, PA, 6janssen Pharmaceuticals, Spring House, PA, 7Janssen Pharmaceuticals, La Jolla, CA, 8Chemistry and Biochemistry, University of California, San Diego, La Jolla, CA, 9Medicine, UCSD, La Jolla, CA

    Background/Purpose: RA FLS display a unique aggressive phenotype with a distinctive DNA methylation profile that marks genes involved with cytokine signaling, cell recruitment, and matrix…
  • Abstract Number: 3120 • 2016 ACR/ARHP Annual Meeting

    RA Net: A Systems Biology Approach to Identify Genes Regulating Pathogenic Pathways in Rheumatoid Arthritis (RA) Fibroblast-like Synoviocytes (FLS)

    Wei Wang1, RIchard Ainsworth2, Richard Stein2, Rizi Ai3 and Gary Firestein4, 1Chemistry and Biochemistry, University of California, San Diego, La Jolla, CA, 2UC San Diego, La Jolla, CA, 3Chemistry and Biochemistry, UC San Diego, La Jolla, CA, 4Medicine, UCSD, La Jolla, CA

    Background/Purpose: Rheumatoid arthritis (RA) remains a significant unmet need despite improved therapy. Defining the interlaced nature of gene regulation and disease pathogenesis using integrative systems…
  • Abstract Number: 3121 • 2016 ACR/ARHP Annual Meeting

    Specific Antibody Subphenotypes in Rheumatoid Arthritis Are Associated with Unique HLA-DRB1 Residues at Position 11

    Chikashi Terao1,2, Boel Brynedal3, Zuomei Chen4, Xia Jiang4, Helga Westerlind4, Monika Hansson5, Linda Mathsson-Alm6,7, Per Johan Jakobsson8, Karl Skriner9, Guy Serre10, Johan Rönnelid7, Leonid Padyukov8, Jane Worthington11, Lars Alfredsson4, Lars Klareskog8 and Soumya Raychaudhuri1,2,11, 1Departments of Genetics and Rheumatology, Brigham and Women'’s Hospital, Harvard Medical School, Boston, MA, 2Program in Medical and Population Genetics, Broad Institute, Boston, MA, 3Section of Epidemiology, Institute of Environmental Medicine, Karolinska Institutet, Karolinska, Sweden, 4Section of Epidemiology, Institute of Environmental Medicine, Karolinska Institutet, Stockholm, Sweden, 5Rheumatology Unit, Department of Medicine, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden, 6Thermo Fisher Scientific, Uppsala, Sweden, 7Department of Immunology Genetics and Pathology,Uppsala University, Uppsala, Sweden, 8Unit of Rheumatology, Department of Medicine, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, 9Humboldt University of Berlin, Berlin, Germany, 10Unité Différenciation Épidermique et Autoimmunité Rhumatoïde, Unité Mixte de Recherche, INSERM, Toulouse, France, 11Arthritis 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:   Antibody to citrullinated peptides/proteins (ACPA) is specific feature of rheumatoid arthritis (RA) and seen in 50 to 70% of RA patients. Amino acid (AA)…
  • Abstract Number: 3122 • 2016 ACR/ARHP Annual Meeting

    Genome-Wide DNA Methylation Association Study of Hand Osteoarthritis

    Michelle S. Yau1,2, Roby Joehanes3, Yi-Hsiang Hsu3, Douglas P. Kiel4 and David T. Felson5, 1Institute for Aging Research, Hebrew SeniorLife, Harvard Medical School, Boston, MA, 2Clinical Epidemiology Research and Training Unit, Boston University School of Medicine, Boston, MA, 3Hebrew SeniorLife, Harvard Medical School, Boston, MA, 4Institute for Aging Research, Institute for Aging Research, Hebrew Senior Life, Harvard Medical School, Boston, MA, USA, Boston, MA, 5Arthritis Research UK Centre for Epidemiology, Institute of Inflammation and Repair, University of Manchester, Manchester, United Kingdom

    Background/Purpose: Heritability of hand osteoarthritis (OA) has been estimated to be as high as 65%.  Despite having a strong genetic component, genome-wide linkage and association…
  • Abstract Number: 3123 • 2016 ACR/ARHP Annual Meeting

    Rare Mediterranean Fever (MEFV) Gene Polymorphisms Are Associated with Ankylosing Spondylitis in Turkish and Iranian Population

    Zhixiu Li1, Servet Akar2, Handan Yarkan3, Pinar Cetin3, Gerçek Can3, Gökce Kenar3, Omer Nuri Pamuk4, Yavuz Pehlivan5, Katie Cremin6, Erika De Guzman1, Jessica Harris1, Ahmad Reza Jamshidi7, Mahdi Vojdanian7, Nooshin Ahmadzadeh7, Mahdi Mahmoudi7, Matthew A. Brown1 and Nurullah Akkoc3, 1Translational Genomics Group, Institute of Health and Biomedical Innovation, Queensland University of Technology, Translational Research Institute, Brisbane, Australia, Brisbane, Australia, 2Department of Rheumatology, İzmir Katip Çelebi University, School of Medicine, İzmir, Turkey, İzmir, Turkey, 3Department of Rheumatology, Dokuz Eylul University, Faculty of Medicine, İzmir, Turkey, İzmir, Turkey, 4Department of Rheumatology, Trakya University Medical Faculty, Edirne, Turkey, Edirne, Turkey, 5Department of Rheumatology, Uludag University Medical Faculty, Bursa, Turkey, Bursa, Turkey, 6The University of Queensland Diamantina Institute, Translational Research Institute, Brisbane, Australia, Brisbane, Australia, 7Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran, Tehran, Iran (Islamic Republic of)

    Background/Purpose: Ankylosing spondylitis (AS) is a highly heritable inflammatory arthritis common in both Turkish and Iranian populations. Familial Mediterranean Fever (FMF) is an autosomal recessive…
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