Abstracts tagged "juvenile dermatomyositis"

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: 1851 • 2018 ACR/ARHP Annual Meeting
Physical Function Trajectories in Children with Juvenile Myositis
Kaveh Ardalan¹, Elizabeth L. Gray², Julia (Jungwha) Lee², Madison L. Wolfe³, Gabrielle A. Morgan⁴ and Lauren M. Pachman⁵, ¹Departments 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, ²Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, ³Creighton University School of Medicine, Omaha, NE, ⁴Cure JM Program of Excellence in Myositis Research, Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, 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
Background/Purpose: Juvenile myositis (JM) is an inflammatory disease that causes muscle weakness, skin rashes, and significant deconditioning. Little is known about long-term resolution of physical…
Abstract Number: 2031 • 2018 ACR/ARHP Annual Meeting
Dysregulated NK Cell PLC
Allison A. Throm¹, Joshua B. Alinger¹, Lauren M. Pachman² and Anthony R. French³, ¹Pediatrics, Washington University St Louis, St Louis, MO, ²Cure 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, ³Pediatric Rheumatology, Washington University St Louis, Saint Louis, MO
Background/Purpose: Juvenile dermatomyositis (JDM) is a debilitating pediatric autoimmune disease manifesting with characteristic rash and proximal muscle weakness. We investigated signaling abnormalities in immune cell…
Abstract Number: 2285 • 2018 ACR/ARHP Annual Meeting
Factors Associated with Corticosteroid Discontinuation, Complete Clinical Response and Remission in Patients with Juvenile Dermatomyositis
Takayuki Kishi^1,2, William Warren-Hicks³, Nastaran Bayat¹, Ira Targoff⁴, Terri H Finkel⁵, Ellen Goldmuntz⁶, Michael Henrickson⁷, Bianca Lang⁸, Andrew Mammen⁹, Lauren M. Pachman¹⁰, Murray Passo¹¹, Terrance P. O'Hanlon¹, Frederick W. Miller¹, Michael Ward⁹ and Lisa G. Rider¹, ¹Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, ²Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, ³EcoStat, Inc., Mebane, NC, ⁴VA Medical Center, University of Oklahoma Health Sciences Center and Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁵Department of Pediatrics, Nemours Children's Health System/ Nemours Children's Hospital, Orlando, FL, ⁶NIAID, NIH, Bethesda, MD, ⁷Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁸Department of Pediatrics, IWK Health Centre and Dalhousie University, Halifax, NS, Canada, ⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁰Cure 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, ¹¹Division of Rheumatology PTD, Medical University of South Carolina, Charleston, SC
Background/Purpose: We examined patients in a large juvenile dermatomyositis (JDM) registry for frequency of and factors associated with final corticosteroid discontinuation (Steroid DC), complete clinical…
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 Ardalan^3,4, 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 Ardalan^3,4, 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 Bollerslev^2,3, Thomas Schwartz^4,5, Berit Flatø^3,6, Ivar Sjaastad^3,4,7 and Helga Sanner^6,8, 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…
Abstract Number: 852 • 2017 ACR/ARHP Annual Meeting
RNAseq Detection of Gene Dysregulation in PBMCs from Juvenile Dermatomyositis, Positive for p155/140 Myositis Specific Antibody
Chiang-Ching Huang¹, Victoria Hans², Dong Xu³, Megan L. Curran^4,5, Gabrielle A. Morgan⁶, Elisha D.O. Roberson⁷ and Lauren M. Pachman^8,9, ¹Biostatistics, Joseph J. Zilber School of Public Health,, Milwaukee, WI, ²CureJM Center of Excellence, Stanly Manne Research Center, Chicago, IL, ³Pediatric Rheumatology, Stanley Manne Research Center, Chicago, IL, ⁴Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵Division of Rheumatology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ⁶Cure JM Program of Excellence in Myositis Research, Chicago, IL, ⁷Depts. of Medicine and Genetics, Division of Rheumatology, Washington University, St. Louis, MO, ⁸Cure 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, ⁹Pediatric Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL
Background/Purpose: Children with Juvenile Dermatomyositis (JDM) have variable responses to the available immunosuppressive drugs, with less than optimal outcomes, making it essential to characterize their…
Abstract Number: 1274 • 2017 ACR/ARHP Annual Meeting
Anti-MDA5 Autoantibodies Associated with Juvenile Dermatomyositis Constitute a Distinct Phenotype in North America
Gulnara Mamyrova¹, Takayuki Kishi², Ira N Targoff³, Rodolfo V Curiel⁴, Frederick W Miller² and Lisa G Rider², ¹Rheumatology, George Washington University School of Medicine and Health Sciences, Washington, DC, ²Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, ³VA Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁴George Washington University School of Medicine and Health Sciences, Washington, DC
Background/Purpose: Anti-MDA5 autoantibodies (Abs) have been associated with clinically amyopathic and classic dermatomyositis (DM), with rapidly progressive interstitial lung disease (ILD) and poor prognosis in…
Abstract Number: 1277 • 2017 ACR/ARHP Annual Meeting
Longitudinal Predictors of Physical Function in Juvenile Myositis
Kaveh Ardalan^1,2, Hannah L. Palac^3,4, Julia (Jungwha) Lee⁴, Madison Wolfe^5,6, Gabrielle A. Morgan^7,8 and Lauren M. Pachman⁹, ¹Departments of Pediatrics and Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, IL, ²Division of Rheumatology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, ³Abbvie, Inc, Chicago, IL, ⁴Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵Division of Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ⁶Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, ⁷Cure JM Program of Excellence in Myositis Research, Chicago, IL, ⁸Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ⁹Cure 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
Background/Purpose: Juvenile myositis (JM) is marked by skin rashes, proximal muscle weakness, and deconditioning causing potentially severe disability. Studies examining long-term physical function in JM…
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,…

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