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  • Abstract Number: 1457 • 2015 ACR/ARHP Annual Meeting

    An Organ Based Diagnostic Approach to Macrophage Activating Syndrome in Children Demonstrates Impaired Reticuloendothelial System Clearance

    King Soon Goh1, Francesca Minoia2, Angelo Ravelli2, Randy Q. Cron3 and Bita Shakoory4, 1Rheumatology, Temple University Hospital, Philadelphia, PA, 2Istituto Giannina Gaslini, Genoa, Italy, 3University of Alabama at Birmingham, Birmingham, AL, 4Rheumatology, George Washington University, Washington, DC

    Background/Purpose: Early recognition and treatment of Macrophage Activating Syndrome (MAS) can improve outcome. Various diagnostic criteria are available to optimize the diagnosis if suspected by…
  • Abstract Number: 1458 • 2015 ACR/ARHP Annual Meeting

    Association of Chronic, Extreme Elevation of Serum IL-18 with the Development of Macrophage Activation Syndrome in a Cohort of Autoinflammatory Disease Patients: A Potential Diagnostic Biomarker?

    Scott Canna1, Adriana Almeida de Jesus2, Yan Huang3, Sushanth Gouni1, Guangpu Shi4, Igal Gery4 and Raphaela Goldbach-Mansky3, 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, 3Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 4National Eye Institute/NIH, Bethesda, MD

    Background/Purpose: Macrophage Activation Syndrome (MAS) is a life-threatening systemic inflammatory syndrome that complicates several rheumatic diseases. Current MAS-related serum biomarkers (ferritin, neopterin, CD163, and CD25)…
  • Abstract Number: 1459 • 2015 ACR/ARHP Annual Meeting

    Racial Comparisons of Children with Idiopathic Uveitis: Results from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry

    Sheila T. Angeles-Han1,2,3, Curtis Travers3, Mindy S. Lo4, C. Egla Rabinovich5, Sampath Prahalad6 and and CARRA Registry Investigators, 1Children's Healthcare of Atlanta, Atlanta, GA, 2Ophthalmology, Emory University School of Medicine, Atlanta, GA, 3Pediatrics, Emory University School of Medicine, Atlanta, GA, 4Medicine/Immunology, Children's Hospital Boston, Boston, MA, 5Duke University Medical Center, Durham, NC, 6Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA

    Background/Purpose: Childhood uveitis can lead to poor visual outcomes. Our data from single center studies suggests that non-Hispanic African American children (NHB) with non-inflammatory uveitis…
  • Abstract Number: 1460 • 2015 ACR/ARHP Annual Meeting

    Association of Vitamin D Receptor Polymorphism with Juvenile Idiopathic Arthritis (JIA)

    Fernanda Falcini1,2, Francesca Marini3, Stefano Stagi4, Gemma Lepri2, Donato Rigante5, Marco Matucci-Cerinic2 and M. L. Brandi3, 1Department of Internal Medicine, Rheumatology Section, Transition Clinic, University of Florence, Firenze, Italy, 2Department of Internal Medicine, Rheumatology Section, Transition Clinic, University of Florence, Italy, Florence, Italy, 3University of Florence, Dept. of Surgery and Translational Medicine, Florence, Italy, 4Pediatric Endocrinology Unit, Anna Meyer Children’s Hospital, University of Florence, Pediatric Endocrinology Unit, Anna Meyer Children’s Hospital, Firenze, Italy, 5Istitute of Pediatrics, Università Cattolica del Sacro Cuore, Rome, Italy

    Background/Purpose:  JIA is the most common chronic arthritis of childhood. Vitamin D is a potential immuno-modulator in many conditions, including autoimmune diseases. Its influence in…
  • Abstract Number: 1461 • 2015 ACR/ARHP Annual Meeting

    Inflammatory Bowel Disease in Children with Systemic Juvenile Idiopathic Arthritis

    Emily Fox1, Joyce Hsu2, Elizabeth Chalom3,4, Sarah Sertial5, KT Park6, Julia F Simard7, Pierre Quartier8, Maria Teresa Terreri9, Kevin Baszis10, Charlotte Borocco11, Sampath Prahalad12, Adam Reinhardt13, Dieneke Schonenberg14, Elizabeth D. Mellins15 and Devy Zisman16, 1Pediatric Rheumatology, Stanford, Palo Alto, CA, 2Pediatric Rheumatology, Stanford University, Palo Alto, CA, 3Pediatrics, St Barnabas Med Center-ACC, Livingston, NJ, 4Pediatric Rheumatology, St. Barnabas Medical Center, West Orange, NJ, 5Children's Hospital of New Jersey, Livingston, NJ, 6Division of Gastroenterology, Stanford University School of Medicine, Stanford, CA, 7Division of Epidemiology, Health Research and Policy Department, and Division of Immunology & Rheumatology, Department of Medicine, Stanford School of Medicine, Stanford, CA, 8Hôpital Necker-Enfants Malades, Paris, France, 9Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, 10Pediatrics, Division of Rheumatology, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, 11Necker Hospital for Sick Children, Paris, France, 12Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, 13Faculty of Physicians of the University of Nebraska Medical Center, College of Medicine, Nebraska, NE, 14Department of Pediatric Rheumatology and Immunology, Emma Children's Hospital, Academic Medical Center, Amsterdam, Netherlands, 15Dept of Pediatrics CCSR, Stanford University Med Ctr, Stanford, CA, 16Rheumatology Unit, Carmel Medical CenterThe Ruth and Bruce Rappaport Faculty of Medicine, Technion, Israel, Haifa, Israel

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease accompanied by systemic symptoms including lymphadenopathy, serositis, and hepatosplenomegaly. Recently, we encountered a patient previously…
  • Abstract Number: 1462 • 2015 ACR/ARHP Annual Meeting

    Farber Disease: Important Differential Diagnostic Information for JIA and Other Inflammatory Arthritis Phenotypes Is Revealed By Data from the Largest Clinical Cohort to Date

    Alexander Solyom1, Boris Huegle2, Bo Magnusson3, Balahan Makay4, Nur Arslan5, John Mitchell6, Pranoot Tanpaiboon7, Norberto Guelbert8, Ratna Puri9, Lawrence Jung10, Giedre Grigelioniene11, Karoline Ehlert12, Michael Beck13, Calogera Simonaro14 and Edward Schuchman15, 1Pediatrics, University of Pecs, Pecs, Hungary, 2Pediatric Rheumatology, German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany, 3Pediatric Rheumatology, Karolinska University Hospital, Stockholm, Sweden, 4Pediatric Rheumatology, Eylul University, Izmir, Turkey, 5Eylul University, Izmir, Turkey, 6Pediatric Endocrinology, Montreal Children's Hospital, Montreal, QC, Canada, 7Genetics and Metabolism, Children's National Medical Center, Washington, DC, 8Metabolic Diseases Section, Children’s Hospital of Cordoba, Cordoba, Argentina, 9Medical Genetics, Sir Ganga Ram Hospital, New Delhi, India, 10Pediatric Rheumatology, Children's National Med Ctr, Washington, DC, 11Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden, 12University Medical Center - Greifswald, Greifswald, Germany, 13Institute of Human Genetics, University Medical Center - Mainz, Mainz, Germany, 14Genetics and Genomic Sciences, Icahn School of Medicine at Mt. Sinai, New York, NY, 15Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY

    Background/Purpose: Farber disease (Farber lipogranulomatosis; acid ceramidase deficiency) is a rare lysosomal storage disorder resulting from the inherited deficiency of the enzyme acid ceramidase due…
  • Abstract Number: 1463 • 2015 ACR/ARHP Annual Meeting

    Anti-Cyclic Citrullinated Peptide Antibody Subclass Phenotypes in Polyarticular Juvenile Idiopathic Arthritis

    Hannah Peckham1, Lauren Bourke2,3, Anna Radziszewska4, Maria Leandro5, Debajit Sen2, Geraldine Cambridge6 and Yiannis Ioannou7,8, 1Adolescent Centre for Rheumatology Research, University College London, London, United Kingdom, 2Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 3Centre for Rheumatology Research, University College London, London, United Kingdom, 4The Rayne Institute, Arthritis Research UK Centre for Adolescent Rheumatology at University College London, Great Ormond Street Hospital and UCLH, University College London, London, United Kingdom, 5Centre for Rheumatology, University College London, London, United Kingdom, 6Centre for Rheumatology, Department of Medicine, University College London, London, United Kingdom, 7Arthritis Research UK Centre for Adolescent Rheumatology at University College London, Great Ormond Street Hospital and UCLH, University College London, London, United Kingdom, 8Centre for Rheumatology Research, University College Hospital London, London, United Kingdom

    Background/Purpose: Raised levels of Rheumatoid Factor (RhF) and antibodies to citrullinated proteins (ACPA), detected in the clinic using combinations of cyclic citrullinated peptides (CCP), are…
  • Abstract Number: 1464 • 2015 ACR/ARHP Annual Meeting

    Assessment of Left Ventricle Functions in Children with Familial Mediterranean Fever with Tissue Doppler and Strain Echocardiography

    Fatih Kelesoglu1, Gafur Dogdu2, Remzi Sarikaya3, Adem Atici3, Serra Karaca4, Zeynep Sarac5, Feyza Tiryaki5, Omer Kumas5, Zehra Bugra6, Sevinc Emre7 and Rukiye Omeroglu8, 1Pediatric Rheumatology, Istanbul Medical School, Pediatric Rheumatology, Istanbul, Turkey, 2Pediatric Cardiology, Istanbul Medical School, Pediatric Cardiology, istanbul, Turkey, 3Cardiology, istanbul Medical School, Cardiology, istanbul, Turkey, 4Pediatric Rheumatology, istanbul Medical School, Pediatric Cardiology, istanbul, Turkey, 5Student, istanbul Medical School, istanbul, Turkey, 6cardiology, istanbul Medical School, Cardiology, istanbul, Turkey, 7Istanbul Medical School, Pediatric Rheumatology, istanbul, Turkey, 8Pediatric Rheumatology, Istanbul Medical School, Pediatric Rheumatology, istanbul, Turkey

    Assessment of Left Ventricle Functions in Children with Familial Mediterranean Fever with Tissue Doppler and Strain Echocardiography Background/Purpose: The effects of Familial Mediterranean Fever (FMF)…
  • Abstract Number: 1465 • 2015 ACR/ARHP Annual Meeting

    Musculoskeletal Anomalies in Children with Trisomy 21

    charlene foley1, Orla G killeen1 and emma Jane macDermott2, 1Our Lady's Children's Hospital Crumlin, National Centre for Paediatric Rheumatology, Dublin, Ireland, 2our Lady's Children's Hospital Crumlin, National Centre for Paediatric Rheumatology, Dublin, Ireland

    Background/Purpose:  Musculoskeletal complications of Trisomy 21 (T21) are common.  Almost all children with T21 have muscle hypotonia and joint laxity.  The combination of this ligamentous…
  • Abstract Number: 1466 • 2015 ACR/ARHP Annual Meeting

    Assessment of Transition Readiness in Adolescents and Young Adults with Chronic Health Conditions

    Paul Jensen1,2, Gabrielle Paul3, Stephanie LaCount4, Brendan Boyle5, Manmohan K. Kamboj6 and Stacy P. Ardoin7, 1Rheumatology, Nationwide Children's Hospital, Columbus, OH, 2Medicine, Ohio State University Medical Center, Columbus, OH, 3College of Medicine, Ohio State University, Columbus, OH, 4Pediatrics, Nationwide Children's Hospital, Columbus, OH, 5Gastroenterology, Nationwide Children's Hospital, Columbus, OH, 6Endocrinology, Nationwide Children's Hospital, Columbus, OH, 7Pediatric & Adult Rheumatology, Ohio State University, Columbus, OH

    Background/Purpose: The transition from pediatric to adult care is a vulnerable period associated with increased morbidity and mortality. Guidelines call for assessing patients’ transition readiness…
  • Abstract Number: 1467 • 2015 ACR/ARHP Annual Meeting

    Health Related Quality of Life Is Reduced in Pediatric Patients with Juvenile Idiopathic Arthritis and Juvenile-Onset Fibromyalgia

    Michal Feldon1, Catherine Donnelly2, Hermine I. Brunner3, Anne Louis Johnson1, Lukasz Itert4, Lisa G Rider5 and Edward H. Giannini3, 1Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Internal Medicine, University of Cincinnati, Cincinnati, OH, 3Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD

    Background/Purpose : A number of validated measures are now available for the evaluation of health related quality of life (HRQOL) in children with rheumatic diseases,…
  • Abstract Number: 1468 • 2015 ACR/ARHP Annual Meeting

    The Proposed Outcome Parameters of the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC) with Uveitis Disability VAS Score Correspond Significantly with Uveitis “Classicaly Assessed” Uveitis Activity

    Ivan Foeldvari1, Sandra Schenck2, Gabriele Simonini3, Cinzia DeLibero4, Gabriele Brumm5, Kaisu Kotaniemi6, Susan Mary Nielsen7, Regitze Bangsgaard7, Irene Pontikaki8, Valeria Maria Gerloni8, Elisabetta Miserocchi9, Vasco Miranda10, Margarida Guedes11, Sheila T. Angeles-Han12, Steven Yeh13, Jordi Anton14, Rosa Bou Torrent15, Carmen García de Vicuña16, Martina Niewerth17 and Arnd Heiligenhaus18, 1Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, 2Deutsches Rheuma-Forschungszentrum, Berlin, Germany, 3Pediatric Rheumatology, Pediatric Rheumatology Unit, Anna Meyer Children's Hospital-University of Florence, Firenze, Italy, 4Ophthalmology Unit, Anna Meyer Children's Hospital, Florence, Italy, 5Klinik und Polyklinikum für Augenheilkunde, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany, 6Ophthalmology Department, Rheumatism Foundation Hospital, Heinola, Finland, 7Rigshospitalet, Copenhagen, Denmark, 8Dipartimento di Reumatologia, Università di Milano - Istituto Gaetano Pini, Milano, Italy, 9Department of Ophthalmology, Scientific Institute San Raffaele, University Vita-Salute, Milan, Italy, 10Centro Hospitalar do Porto, Porto, Portugal, 11Hospital de Santo António - Centro Hospitalar do Porto, Porto, Portugal, 12Pediatrics, Emory Children's Center, Atlanta, GA, 13Ophthalmology, Emory University School of Medicine, Atlanta, GA, 14Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, 15Pediatrics Department, Pediatric Rheumatology Unit, Universitat de Barcelona, Hospital Sant Joan de Deu, Barcelona, Spain, 16Universitat de Barcelona, Hospital Sant Joan de Deu, Barcelona, Spain, 17Epidemiology, German Rheumatism Research Centre, Berlin, Germany, 18Department of Ophthalmology at St. Franziskus Hospital Muenster, University of Duisberg-Essen, Muenster, Germany

    Background/Purpose: Juvenile idiopathic arthritis (JIA) associated uveitis is one of the most severe comorbidities of JIA and occurs in around 10% of JIA patients.  There…
  • Abstract Number: 1469 • 2015 ACR/ARHP Annual Meeting

    A Comparison of Pain and Disability, and Their Association Between Juvenile Primary Fibromyalgia Syndrome and Pediatric Rheumatic Diseases: Results from the Childhood Arthritis and Rheumatology Research Alliance Registry

    Jennifer E. Weiss1 and Mark Connelly2, 1Hackensack Univ Med Ctr, Hackensack, NJ, 2Psychology, University of Kansas Medical Center, Kansas City, KS

    Background/Purpose: We aim to determine the extent to which pain severity differs between patients with JPFS and other rheumatic diseases and if the degree of…
  • Abstract Number: 1470 • 2015 ACR/ARHP Annual Meeting

    A Population-Based Study of Outcomes of Patients with Juvenile Idiopathic Arthritis (JIA) Compared to Non-JIA Subjects    

    Megan L. Krause1, J.A. Zamora-Legoff2, Cynthia S. Crowson3, Thomas Mason II1, Theresa Wampler Muskardin2 and Eric L. Matteson4, 1Rheumatology, Mayo Clinic, Rochester, MN, 2Division of Rheumatology, Mayo Clinic, Rochester, MN, 3Health Sciences Research, Mayo Clinic, Rochester, MN, 4Division of Rheumatology, Department of Internal Medicine and Department of Health Sciences Research, Mayo Clinic, Rochester, MN

    Background/Purpose: The impact of juvenile idiopathic arthritis (JIA) is not confined to joint involvement in children but rather widespread effects extending to adulthood.  This study…
  • Abstract Number: 1471 • 2015 ACR/ARHP Annual Meeting

    Phenotypic Differences Between HLA-B27 Positive and Negative Children with Enthesitis-Related Arthritis

    Sabrina Gmuca1, Timothy Brandon2, Rui Xiao3, Ilaria Pagnini4, Tracey B. Wright5, Timothy Beukelman6, Esi Morgan-DeWitt7 and Pamela F. Weiss8, 1Pediatric Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA, 2Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, 3Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PA, 4Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, 5Pediatrics/Rheumatology, UT Southwestern Medical Center, Dallas, TX, 6Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 7Pediatric rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 8Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA

    Background/Purpose: The association of HLA-B27 with spondyloarthritis is well-established. Under the International League of Associations for Rheumatology (ILAR) criteria most children with spondyloarthritis are classified…
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