ACR Meeting Abstracts

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  • Abstract Number: 317 • 2014 ACR/ARHP Annual Meeting

    Myeloid Related Proteins 8 and 14 (MRP 8/14) – Potential Biomarkers of Disease Activity of Arthritis in Children with Trisomy 21

    Charlene Foley, Orla Killeen and Emma Jane MacDermott, The National Centre for Paediatric Rheumatology, Dublin, Ireland

    Background/Purpose JIA is an umbrella term used to describe a heterogeneous group of diseases.To date no specific markers exist in clinical practice to predict disease…
  • Abstract Number: 315 • 2014 ACR/ARHP Annual Meeting

    NLRC4-Related Macrophage Activation Syndrome (NLRC4-MAS): A Novel Primary Autoinflammatory Syndrome Caused By Activating Mutations in NLRC4

    Scott Canna1, Adriana Almeida de Jesus2, Sushanth Gouni1, Stephen Brooks3, Kristien J. Zaal4, Bernadette Marrero5, Yin Liu6, Michael Dimattia7, Gina A. Montealegre Sanchez6, Hanna Kim6, Dawn C. Chapelle6, Nicole Plass6, Yan Huang6, Angelique Biancotto8, J. Alex Duncan9, Susanne Benseler10, John J. O'Shea1, Alexei A. Grom11, Zuoming Deng12, Ronald Laxer13 and Raphaela Goldbach-Mansky14, 1Molecular Immunology and Inflammation Branch, NIAMS/NIH, Bethesda, MD, 2Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 3NIAMS/NIH, Bethesda, MD, 4Light Imaging Section, NIAMS/NIH, Bethesda, MD, 5Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, MD, 6Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 7Laboratory of Structural Biology, NIAMS/NIH, Bethesda, MD, 8Center for Human Immunology, Autoimmunity and Inflammation, NHLBI/NIH, Bethesda, MD, 9Infectious Diseases, University of North Carolina School of Medicine, Chapel Hill, NC, 10Alberta Children's Hospital Research Institute/University of Calgary, Calgary, AB, Canada, 11Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 12Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 13Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, 14Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD

    Background/Purpose: Macrophage Activation Syndrome (MAS) is a life-threatening systemic inflammatory complication of many rheumatic diseases and its causes are unknown. While genetic defects causing impaired…
  • Abstract Number: 314 • 2014 ACR/ARHP Annual Meeting

    Next Generation Sequencing Reveals Restriction of the Treg Cell Repertoire and an Abundance of Shared Synovial Treg Clonotypes in JIA

    Lauren A. Henderson1, Stefano Volpi1, Francesco Frugoni2, Susan Kim1, Robert P. Sundel1, Fatma Dedeoglu3, Mindy S. Lo1, Erin Janssen1, Melissa M. Hazen1, Mary Beth Son1, Ronald Mathieu4, David Zurakowski5, Robert C. Fuhlbrigge1, Jolan E. Walter6, Yu Nee Lee1, Peter A. Nigrovic1 and Luigi D Notarangelo7, 1Division of Immunology, Boston Children's Hospital, Boston, MA, 2Immunology, Boston Children's Hospital, Boston, MA, 3Rheumatology, Boston Children's Hospital, Boston, MA, 4Division of Hematology and Oncology, Boston Children's Hospital, Boston, MA, 5Departments of Anesthesia and Surgery, Boston Children’s Hospital, Boston, MA, 6Division of Pediatric Allergy/Immunology, Massachusetts General Hospital for Children, Bpstpm, MA, 7Division of Immunology and The Manton Center for Orphan Disease Research, Boston Children's Hospital, Boston, MA

    Background/Purpose Regulatory T cell (Treg) dysfunction has been documented in juvenile idiopathic arthritis (JIA), but the basis for this lapse in suppressive capacity is incompletely…
  • Abstract Number: 313 • 2014 ACR/ARHP Annual Meeting

    Elevated Cardiovascular Disease Burden and Inflammatory Biomarker Levels in Adults with Juvenile Idiopathic Arthritis

    Siobhan Crittenden1, Elizabeth Coulson1,2, Vijay Kunadian3,4,5, Wan-Fai Ng1,2,6 and H. E. Foster7,8,9, 1Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle upon Tyne, United Kingdom, 2Rheumatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom, 3Faculty of Medical Sciences, Institute of Cellular Medicine, Newcastle upon Tyne, United Kingdom, 4Cardiology, Freeman Hospital, Newcastle upon Tyne, United Kingdom, 5Cardiology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom, 6Musculoskeletal Directorate, The Freeman Hospital, Newcastle upon Tyne, United Kingdom, 7Paediatric Rheumatology, Newcastle Hospitals NHS Trust and Great North Children's Hospital, Newcastle Upon Tyne, United Kingdom, 8Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle Upon Tyne, United Kingdom, 9Paediatric Rheumatology, Royal Victoria Infirmary, Newcastle upon Tyne, United Kingdom

    Background/Purpose: Rheumatoid Arthritis (RA) is associated with a 50 % greater cardiovascular mortality rate than the general population, attributable to the increased prevalence of traditional…
  • Abstract Number: 312 • 2014 ACR/ARHP Annual Meeting

    Mutations in the MTHFR Gene Are Not Associated with Methotrexate Intolerance in Patients with Juvenile Idiopathic Arthritis

    Andrea Scheuern1, Nadine Fischer2, Johannes-Peter Haas1 and Boris Hugle1, 1German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany, 2Research, German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany

    Background/Purpose:   Methotrexate (MTX) is the drug used most frequently in the therapy of juvenile idiopathic arthritis (JIA). However, long-term treatment in children frequently leads…
  • Abstract Number: 311 • 2014 ACR/ARHP Annual Meeting

    Inhibition of Natural Killer (NK) Cell Cytotoxicity By Interleukin-6 (IL-6): Implications for the Pathogenesis of Macrophage Activation Syndrome

    Loredana Cifaldi1, Giusi Prencipe2, Ivan Caiello2, Claudia Bracaglia2, Raffaele Strippoli3 and Fabrizio De Benedetti Sr.2, 1Paediatric Haematology/Oncology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, 2Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, 3Cellular Biotechnologies and Haematology, Sapienza Rome University, Rome, Italy

    Background/Purpose: MAS occurs frequently in patients with active systemic juvenile idiopathic arthritis(s-JIA) and because of the similarities with Haemophagocytic Lymphohistiocytosis (HLH) is classified among secondary…
  • Abstract Number: 310 • 2014 ACR/ARHP Annual Meeting

    Differential Expression of microRNA in Monocytes from Children with Systemic Juvenile Idiopathic Arthritis: Implications for Polarized Phenotype

    Grant Schulert1, Ndate Fall2, Nan Shen3 and Alexei Grom4, 1Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 3Rheumatology, Shanghai Institutes for Biological Sciences Chinese Academy of Sciences & Shanghai Jiao Tong University School of Medicine, Shanghai, OH, China, 4Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease of childhood, and the predominant effector cells are mononuclear phagocytes rather than lymphocytes as in…
  • Abstract Number: 309 • 2014 ACR/ARHP Annual Meeting

    Clinical Significance of Cytokine Profile with Interleukin-18 and -6 in Systemic Juvenile Idiopathic Arthritis

    Masaki Shimizu, Natsumi Inoue, Yuko Tasaki, Sayaka Ishikawa, Kazuyuki Ueno and Akihiro Yachie, Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University, Kanazawa, Japan

    Background/Purpose  Innate proinflammatory cytokines interleukin (IL)-6 and IL-18 are critical for perpetuating the inflammatory processes in systemic juvenile idiopathic arthritis (s-JIA) and macrophage activation syndrome…
  • Abstract Number: 307 • 2014 ACR/ARHP Annual Meeting

    Sibling Exposure and Risk of Juvenile Idiopathic Arthritis

    Jessica Miller1, Anne-Louise Ponsonby2,3, Angela Pezic2, Jonathan Akikusa4, Roger Allen4, Jane Munro4 and Justine Ellis5,6, 1Murdoch Childrens Research Institute, Parkville, Australia, 2Environmental & Genetic Epidemiology Research, Murdoch Childrens Research Institute, Parkville, Australia, 3The University of Melbourne, Parkville, Australia, 4Rheumatology, Royal Children's Hospital, Parkville, Australia, 5Genes, Environment, and Complex Disease, Murdoch Childrens Research Institute, Parkville, Australia, 6Department of Paediatrics, The University of Melbourne, Parkville, Australia

    Background/Purpose: Susceptibility to juvenile idiopathic arthritis (JIA) is presumed to be determined by the interplay of genes and environment. Our understanding of the genetic basis…
  • Abstract Number: 308 • 2014 ACR/ARHP Annual Meeting

    Tenascin-C, a TLR4 Ligand Levels in Enthesitis Related Arthritis Category of Juvenile Idiopathic Arthritis: Cross-Sectional and Longitudinal Study

    Anuj Shukla1, Priyanka Gaur2 and Amita Aggarwal1, 1Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, 2Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

    Background/Purpose Tenascin-C (TNC) is an extracellular matrix glycoprotein, which binds to TLR4 and leads to its activation. Monocytes of children with Enthesitis related arthritis (ERA)…
  • Abstract Number: 306 • 2014 ACR/ARHP Annual Meeting

    Association of Kawasaki Disease with Tropospheric Winds in Central Chile: Is Wind-Borne Desert Dust a Risk Factor? 

    Arturo Borzutzky1,2, Alvaro García3, Rodrigo Hoyos-Bachiloglu1,2 and Hector Jorquera4,5, 1Pediatrics, Pontificia Universidad Católica de Chile School of Medicine, Santiago, Chile, 2Millennium Institute on Immunology and Immunotherapy, Pontificia Universidad Católica de Chile, Santiago, Chile, 3Pontificia Universidad Católica de Chile School of Medicine, Santiago, Chile, 4Department of Chemical and Bioprocess Engineering, Pontificia Universidad Católica de Chile School of Engineering, Santiago, Chile, 5Centro de Desarrollo Urbano Sustentable (CEDEUS), Santiago, Chile

    Background/Purpose: Kawasaki disease (KD) has been reported to have seasonal variations in many different countries, as well as geographical and temporal clustering. It has been…
  • Abstract Number: 305 • 2014 ACR/ARHP Annual Meeting

    Understanding the Molecular Pathogenesis of and Response to Canakinumab Treatment in TNF Receptor-Associated Periodic Syndrome  By Gene Expression Profiling of Whole Blood from Patients

    R. Torene1, M. Gattorno2, H. Lachmann3, L. Obici4, A. Meini5, V. Tormey6, R. Caorsi7, L. Baeriswyl8, U. Affentranger8, S. Starck-Schwertz8, M. Letzkus8, N. Hartmann8, K. Abrams9 and N. Nirmala1, 1Novartis Institutes for BioMedical Research, Cambridge, MA, 2Pediatric Rheumatology, G. Gaslini Institute, Genova, Italy, 3Royal Free Hospital, London, United Kingdom, 4Amyloid Centre, IRCCS Policlinico San Matteo, Pavia, Italy, 5Pediatric Clinic, Spedali Civili, Brescia, Italy, 6Galway University Hospitals, Galway, Ireland, 7Second Division of Paediatrics, G. Gaslini Institute, Genova, Italy, 8Novartis Institutes for BioMedical Research, Basel, Switzerland, 9Novartis Pharmaceutical Corporation, East Hanover, NJ

    Background/Purpose: TNF receptor-associated periodic syndrome (TRAPS) is an autoinflammatory disease causing unprovoked fevers, myalgia, abdominal pain, rash, headaches, and, in severe cases, AA amyloidosis. It…
  • Abstract Number: 304 • 2014 ACR/ARHP Annual Meeting

    Juvenile Rheumatoid Arthritis and Future Risk for Cardiovascular Disease; A Multicenter Population-Based Study

    Jason Anderson1, Katelyn Anderson1, Hanne Aulie2, Cynthia S. Crowson3, Thomas Mason II4, Stacy P. Ardoin5, Ann M. Reed6 and Berit Flato7, 1Mayo Clinic, Rochester, MN, 2Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, 3Health Sciences Research, Mayo Clinic, Rochester, MN, 4Division of Rheumatology - Department of Medicine, Mayo Clinic, Rochester, MN, 5Pediatric & Adult Rheumatology, Ohio State University College of Medicine, Columbus, OH, 6Rheumatology, Mayo Clinic, Rochester, MN, 7Department of Rheumatology, Oslo University Hospital, Oslo, Norway

    Background/Purpose: Several studies suggest an increased frequency of cardiovascular disease (CVD) in patients with rheumatoid arthritis, but little is known about CVD risk in patients…
  • Abstract Number: 288 • 2014 ACR/ARHP Annual Meeting

    Establishing Clinical Meaning and Defining Important Differences in Patient Reported Outcome Measures of Physical Function, Fatigue and Pain Interference in Juvenile Idiopathic Arthritis

    Esi M. Morgan DeWitt1, Bin Huang2,3, Kimberly Barnett4, Adam Carle5, Constance Mara6 and Karon Cook7, 1Department of Pediatrics, Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center/University of Cincinnati School of Medicine, Cincinnati, OH, 3Pediatrics, University of Cincinnati School of Medicine, Cincinnati, OH, 4Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 6James M. Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 7Center for Patient-Centered Outcomes - Institute for Public Health and Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL

    Background/Purpose Patient reported outcome measures (PROs) are used increasingly in clinical care. A framework to interpret scores according to degree of clinical severity would enhance…
  • Abstract Number: 287 • 2014 ACR/ARHP Annual Meeting

    Role of Joint Status in Decreased Accelerometer-Assessed Daily Physical Activity in Juvenile Idiopathic Arthritis

    Mette Noergaard1, Marinka Twilt2 and Troels Herlin2, 1Department of Physiotherapy, Aarhus University Hospital, Aarhus, Denmark, 2Pediatric Rheumatology Clinic, Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark

    Background/Purpose Juvenile Idiopathic Arthritis (JIA) is often associated with decreased physical activity (PA). Although improved disease control has opened up the possibilities for JIA-children to…
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