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  • Abstract Number: 1453 • 2015 ACR/ARHP Annual Meeting

    Surveillance of Periodic Fever Syndromes in Canada

    Paul Dancey1,2, Susanne Benseler3, Marco Gattorno4, Anne K Junker5, Ronald Laxer6, Paivi Miettunen7 and Lesley Ann Turner8, 1Health Science Centre, Memorial University of Newfoundland, St Johns, NF, Canada, 2Pediatrics, Janeway Children's Hospital, St. John's, NF, Canada, 3Pediatrics/Alberta Children's Hospital, Department of Pediatrics/University of Calgary, Calgary, AB, Canada, 4Pediatry, G. Gaslini Institute, Genova, Italy, 5University of British Columbia, Vancouver, BC, Canada, 6The Hospital for Sick Children, Toronto, ON, Canada, 7University of Calgary, Calgary, AB, Canada, 8Memorial University, St. John's, NF, Canada

    Background/Purpose: To estimate the incidence of periodic fever syndromes in the Canadian paediatric population, to describe the patterns of presentation, and to raise awareness in…
  • Abstract Number: 1454 • 2015 ACR/ARHP Annual Meeting

    Familial Mediterranean Fever in Childhood: a Single Center Experience

    Kenan Barut1, Ada Bulut Sinoplu2, Gozde Yucel3, Gizem Pamuk2, Amra Adrovic4, Sezgin Sahin4 and Ozgur Kasapcopur5, 1Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University., Fellow in Pediatric Rheumatology, Istanbul, Turkey, 2Department of Pediatric Rheumatology, Cerrahpaşa Medical School, Istanbul University., Resident in Pediatrics, Istanbul, Turkey, 3Department of Pediatric Rheumatology, CerrahpaÅŸa Medical School, Istanbul University., Resident in Pediatrics, Istanbul, Turkey, 4Department of Pediatric Rheumatology, Cerrahpaşa Medical School, Istanbul University., Fellow in Pediatric Rheumatology, Istanbul, Turkey, 5Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Professor of Pediatric Rheumatology, Istanbul, Turkey

    Background/Purpose: Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disease which is clinically manifested with periodic episodes of fever, polyserositis and arthritis.  The…
  • Abstract Number: 1455 • 2015 ACR/ARHP Annual Meeting

    Juvenile Spondyloarthropathies: a Single Center Experience

    Kenan Barut1, Metin Sezen2, Sezgin Sahin3, Amra Adrovic3, Serdal Ugurlu4, Huri Ozdogan5 and Ozgur Kasapcopur6, 1Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University., Fellow in Pediatric Rheumatology, Istanbul, Turkey, 2Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Istanbul, Turkey, 3Department of Pediatric Rheumatology, Cerrahpaşa Medical School, Istanbul University., Fellow in Pediatric Rheumatology, Istanbul, Turkey, 4Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey, 5Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 6Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Professor of Pediatric Rheumatology, Istanbul, Turkey

    Background/Purpose: :  Juvenile spondyloarthropathies (JSpA) are a group of chronic childhood rheumatic diseases which emerge especially before 16 and after 6 years-old. While enthesitis and…
  • Abstract Number: 1456 • 2015 ACR/ARHP Annual Meeting

    Macrophage Activation Syndrome and Familial Hemophagocytic Lymphohistiocytosis: Is Their Clinical Phenotype Really Similar?

    Francesca Minoia1, AnnaCarin Horne2, Sergio Davì1, Francesca Bovis1, Silvia Rosina1, Kai Lehmberg3, Sheila Weitzman4, Antonella Insalaco5, Carine Wouters6, Susan Shenoi7, Graciela Espada8, Seza Ozen9, Jordi Anton10, Raju Khubchandani11, Ricardo Russo12, Nicolino Ruperto13, Alberto Martini1, Randy Q. Cron14 and Angelo Ravelli1, 1Istituto Giannina Gaslini, Genoa, Italy, 2Karolinska University Hospital Solna, Stockholm, Sweden, 3University Medical Center, Hamburg, Germany, 4The Hospital for Sick Children, Toronto, ON, Canada, 5Ospedale Pediatrico Bambino Gesù, Rome, Italy, 6University Hospital Gasthuisberg, Leuven, Belgium, 7Seattle Children's Hospital, Seattle, WA, 8Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina, 9Hacettepe University, Ankara, Turkey, 10Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, 11Jaslok Hospital and Research Center, Mumbai, India, 12Hospital de Pediatria Garrahan, Buenos Aires, Argentina, 13Istituto G. Gaslini, Pediatria II, PRINTO, Genoa, Italy, 14University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Macrophage activations syndrome (MAS) is a potentially life-threatening complication of systemic juvenile idiopathic arthritis (sJIA). It is common view that sJIA associated MAS bears…
  • 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,…
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