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Abstracts tagged "juvenile dermatomyositis"

  • Abstract Number: 480 • 2018 ACR/ARHP Annual Meeting

    Analyze Myositis with Ultrasound and Exercise (AMUSE) Kids- Initial Analysis of Longitudinal Data

    Laura Tasan1, Emily Brunner2, Judy Squires3, Rohit Aggarwal4, Chester V. Oddis4, Christina K. Zigler5, Kaila Schollaert-Fitch6, Emily Mirizio7 and Kathryn S. Torok2, 1Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 2Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 3Radiology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 4Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh/University of Pittsburgh Medical Center, Pittsburgh, PA, 5University of Pittsburgh Med Ctr, Pittsburgh, PA, 6Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, 7Peds Rheum, University of Pittsburgh Med Ctr, Pittsburgh, PA

    Background/Purpose:  There is an unmet need for more objective disease outcome measures in Juvenile Myositis (JM) patients.  This pilot study sought to test the reliability,…
  • Abstract Number: 481 • 2018 ACR/ARHP Annual Meeting

    Stepping It up: The Use of Physical Activity Monitors As an Outcome Measure in Juvenile Myositis

    Emily Brunner1, Laura Tasan2, Kathryn S. Torok3, Bonny Rockette-Wagner4, Christina K. Zigler5, Kaila Schollaert-Fitch3, Diane Koontz6, Chester V. Oddis7 and Rohit Aggarwal8, 1Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 2Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, 3Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, 4University of Pittsburgh Medical Center, Pittsburgh, PA, 5Physical Medicine & Rehabilitation, University of Pittsburgh, Pittsburgh, PA, 6Internal Medicine Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, 7Division of Rheumatology and Clinical Immunology, Department of Medicine, Unviersity of Pittsburgh/University of Pittsburgh Medical Center, Pittsburgh, PA, 8Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh/University of Pittsburgh Medical Center, Pittsburgh, PA

    Background/Purpose: The use of physical activity monitors (PAM), which objectively quantify free-living movement, may enhance assessment of disease activity in juvenile myositis (JM) clinical trials…
  • Abstract Number: 1332 • 2018 ACR/ARHP Annual Meeting

    Proteomic Discovery Analysis Identifies Unique Proteins and Pathways Correlating with Different Clinical Activity and Damage Measures in Juvenile Dermatomyositis (JDM)

    Hanna Kim1, Angelique Biancotto2, Foo Cheung2, Terrance P. O'Hanlon3, Yan Huang4, Frederick W. Miller3, Raphaela Goldbach-Mansky4 and Lisa G. Rider5, 1Pediatric Translational Research Branch, NIAMS, NIH, Bethesda, MD, 2Center for Human Immunology Autoimmunity and Inflammation (CHI), NIAID, NIH, Bethesda, MD, 3Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 4Translational Autoinflammatory Disease Section (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, 5Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

    Background/Purpose: JDM is a complex heterogeneous autoimmune disease.  To define biomarkers and better understand JDM pathogenesis, aptamer-based proteomic technology was used to mine the serum…
  • Abstract Number: 1347 • 2018 ACR/ARHP Annual Meeting

    A Semi-Quantitative Whole Body Magnetic Resonance Imaging Assessment Tool to Define Musculoskeletal Abnormalities in Patients with Idiopathic Inflammatory Myopathies

    Sara Faghihi-Kashani1, Lisa G. Rider2, David Bluemke3, Ashkan Malayeri4, Evrim Turkbey4, Joseph Shrader5, John McGrath6, Elizabeth Jones7, Jamie Marco8, Frederick W. Miller9 and Adam Schiffenbauer10, 1Environmental Autoimmunity Group, National Institute of Environmental Health, Bethesda, MD, 2Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, 3Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI, 4Radiology and Imaging Sciences, National Institutes of Health, Bethesda, MD, 5Rehabilitation Medicine, National Institutes of Health, Bethesda, MD, 6Social and Scientific Systems, Inc., Durham, NC, 7Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, MD, 8Radiology and Imaging Sciences National Institutes of Health, Bethesda, MD, 9Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 10NIEHS, NIH, Bethesda, MD

    Background/Purpose: There is a lack of standardized methodology for assessing whole body MRI (WBMRI) in idiopathic inflammatory myopathy (IIM) patients. This leads to difficulty in…
  • Abstract Number: 1402 • 2018 ACR/ARHP Annual Meeting

    The Relationship of Pain, Fatigue and Emotional Distress with Quality of Life in Juvenile Myositis

    Kyle J. Fahey1, Elizabeth L. Gray2, Rowland W. Chang3, David Cella4, Lauren M. Pachman5 and Kaveh Ardalan6, 1Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, 2Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, 3Preventive Medicine, Medicine, and Physical Medicine & Rehabilitation, Northwestern University Feinberg School of Medicine, Chicago, IL, 4Departments of Medical Social Sciences, Neurology, Pediatrics, Preventive Medicine, and Psychiatry and Behavioral Sciences,, Northwestern University Feinberg School of Medicine, Chicago, IL, 5Cure 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, 6Departments of Pediatrics and Medical Social Sciences, Division of Rheumatology, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL

    Background/Purpose: Juvenile Myositis (JM) is an autoimmune disease that negatively impacts quality of life (QoL) outcomes via muscle weakness and vasculopathic rashes. The relative contribution…
  • Abstract Number: 1279 • 2017 ACR/ARHP Annual Meeting

    Risk Factors for Achieving a Drug-Free Remission in Patients with Juvenile Dermatomyositis

    Tomokazu Nagakura1, Takuma Ito2, Masateru Kusuda2, Tuyoshi Yamatou2, Tomohiro Kubota2, Yuuichi Yamasaki2, Yukiko Nonaka2, Tomoko Takezaki2, Harumi Akaike3, Yasuhito Nerome2, Hiroyuki Imanaka2 and Syuji Takei4, 1Pediatrics, The Holy Mother of Grace Hospital Home for Children and Persons with Severe Motor and Intellectual Disabilities, Usuki, Japan, Usuki, Japan, 2Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, 3Department of Pediatrics, Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, 4Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshma, Japan

    Background/Purpose: Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children. Approximately one-third of JDM patients attain drug-free remission (DFR) without relapse; however,…
  • Abstract Number: 2137 • 2017 ACR/ARHP Annual Meeting

    Clinical Factors Associated with Long-Term Damage and Calcinosis in an Adult-Age Referral Population of Juvenile Myositis Patients

    Vladislav Tsaltskan1, Annette Aldous2, Sam Serafi1, Heidi Sami1, Gulnara Mamyrova1, Frederick W Miller3, Sam Simmens2, Rodolfo Curiel1, Olcay Y. Jones4 and Lisa G Rider3, 1Department of Rheumatology, George Washington University, Washington, DC, 2Department of Epidemiology and Biostatistics, George Washington University Milken Institute School of Public Health, Washington, DC, 3Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, 4Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, MD

    Background/Purpose:  Juvenile idiopathic inflammatory myopathies (JIIM) are rare, autoimmune chronic muscle diseases of childhood with significant potential long-term morbidity. In this study we investigate associations…
  • Abstract Number: 2142 • 2017 ACR/ARHP Annual Meeting

    Autoantibodies Recognizing Cytosolic 5’-Nucleotidase 1A Are Associated with More Severe Disease in Patients with Juvenile Myositis

    Richard Yeker1, Iago Pinal-Fernandez2, Takayuki Kishi3, Ira N. Targoff4, Frederick W Miller3, Lisa G Rider3 and Andrew Mammen5, 1National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 2NIAMS, NIH, Bethesda, MD, 3Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, 4VA Medical Center, University of Oklahoma Health Sciences Center, Oklahoma Medical Research Foundation, Oklahoma City, OK, 5Muscle Diseases Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases. National Institutes of Health, Bethesda, MD

    Background/Purpose: Autoantibodies recognizing cytosolic 5’-nucleotidase 1A (NT5C1A) are present in the sera of adults with myositis and other autoimmune diseases. They are especially prevalent in…
  • Abstract Number: 2259 • 2017 ACR/ARHP Annual Meeting

    Quality of Life Evaluation in New Onset Juvenile Dermatomyositis Patients from the Printo Trial

    Andressa Guariento1, Gabriella Giancane2, Elena Fueri1, Francesco Zulian3, Angelo Ravelli4, Bo Magnusson3, Tadej Avcin3, Fabrizia Corona3, Valeria Gerloni1, Claudia Bracaglia1, Rolando Cimaz1, Antonella Meini2, Silvana Martino3, Anne Pagnier1, Michel Rodiere3, Christine Soler3, Valda Stanevicha3, Rebecca ten Cate5, Jelena Vojinovic3, Simona Angioloni1, Luca Villa1, Michele Pesce1, Irene Gregorini1, Chiara Pallotti1, Alberto Martini6, Angela Pistorio3 and Nicola Ruperto3, 1Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genova, Italy, 2Pediatria II, Reumatologia - PRINTO, Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genova, Italy, 3Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genoa, Italy, 4University of Genova, IRCCS Istituto Giannina Gaslini, Genova, Italy, 5Pediatric Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 6Istituto Giannina Gaslini, Genoa, Italy

    Background/Purpose: Juvenile dermatomyositis (JDM) is the most common clinical pediatric idiopathic inflammatory myopathy and it may severely compromise the quality of life of affected patients.…
  • 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: 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: 2895 • 2017 ACR/ARHP Annual Meeting

    Predictors of Hospitalization, Length of Stay and Costs of Care Among Children with Juvenile Dermatomyositis in the United States

    Michael C. Kwa1, Jonathan I. Silverberg2 and Kaveh Ardalan3,4, 1Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 2Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, 3Departments of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, 4Division of Rheumatology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL

    Background/Purpose: Juvenile dermatomyositis (JDM) is a rare autoimmune disease that causes significant morbidity and quality of life impairment. Little is known about JDM inpatient burden…
  • Abstract Number: 2960 • 2017 ACR/ARHP Annual Meeting

    Cardiovascular and Cerebrovascular Comorbidities of Juvenile Dermatomyositis in United States Children

    Jonathan I. Silverberg1, Lauren Kwa2, Michael C. Kwa2, Anne Laumann2 and Kaveh Ardalan3,4, 1Dermatology, Preventive Medicine and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, 2Dermatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 3Departments of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, 4Division of Rheumatology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL

    Background/Purpose: Juvenile dermatomyositis (JDM) is an autoimmune disease that causes vasculopathy and inflammation of skin and muscles. Previous studies in adult dermatomyositis suggest increased risks…
  • Abstract Number: 2961 • 2017 ACR/ARHP Annual Meeting

    Body Composition and Myokine Levels in Juvenile Dermatomyositis and Associations with Physical Function

    Birgit Nomeland Witczak1, Kristin Godang2, Jens Bollerslev2,3, Thomas Schwartz4,5, Berit Flatø3,6, Ivar Sjaastad3,4,7 and Helga Sanner6,8, 1Oslo University Hospital, Institute for Experimental Medical Research, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 2Section of Specialized Endocrinology, Department of Endocrinology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Oslo, Norway, 3Institute for Clinical Medicine, University of Oslo, Oslo, Norway, Oslo, Norway, 4Institute for Experimental Medical Research, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 5Department of Infectious Diseases, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 6Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Oslo, Norway, 7Department of Cardiology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 8Norwegian National Advisory Unit on Rheumatic Diseases in Children and Adolescents, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Oslo, Norway

    Background/Purpose: JDM presents with proximal muscle weakness and atrophy is frequent. Still, body composition (BC) in JDM has not been widely studied, but is known…
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