Abstracts tagged "juvenile dermatomyositis"

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: 477 • 2018 ACR/ARHP Annual Meeting
Early Treatment with Intravenous Pulse Methylprednisolone or Methotrexate Is Associated with Decreased Medication Requirements at 12 and 24 Months in Patients with Juvenile Dermatomyositis: A Propensity Score Analysis
Takayuki Kishi^1,2, Jesse Wilkerson³, Marjo Smith³, Nastaran Bayat¹, Michael Henrickson⁴, Bianca Lang⁵, Murray Passo⁶, Frederick W. Miller¹, Michael Ward⁷ 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, ³Social & Scientific Systems, Inc., Durham, NC, ⁴Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵Department of Pediatrics, IWK Health Centre and Dalhousie University, Halifax, NS, Canada, ⁶Division of Rheumatology PTD, Medical University of South Carolina, Charleston, SC, ⁷National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD
Background/Purpose: Daily oral prednisone has been the main therapy in juvenile dermatomyositis (JDM), and combination therapy with methotrexate (MTX) introduced soon after diagnosis has been…
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: 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,…
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…

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