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Abstracts tagged "juvenile dermatomyositis"

  • 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: 84 • 2017 Pediatric Rheumatology Symposium

    Predictors of Corticosteroid Discontinuation, Complete Clinical Response and Remission in Patients with Juvenile Dermatomyositis

    Takayuki Kishi1, William Warren-Hicks2, Michael Ward3, Nastaran Bayat1, Lan Wu1, Gulnara Mamyrova4, Ira N. Targoff5, Frederick Miller1, Lisa G. Rider1 and the Childhood Myositis Heterogeneity Study Group, 1Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institute of Health, Bethesda, MD, 2EcoStat, Inc., Mebane, NC, 3National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 4Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC, 5VA Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, OK

    Background/Purpose:  Factors affecting treatment (Rx) responses in juvenile dermatomyositis (JDM) are not well understood.  We examined a large JDM registry for predictors of excellent Rx…
  • Abstract Number: 16 • 2017 Pediatric Rheumatology Symposium

    Novel Serum Broad-Based Proteomic Discovery Analysis Identifies Proteins and Pathways Dysregulated in Juvenile Dermatomyositis (JDM) Myositis Autoantibody Groups

    Hanna Kim1, Angélique Biancotto2, Foo Cheung3, Terrance P. O'Hanlon4, Ira N. Targoff5, Yan Huang6, Frederick 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), 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: 86 • 2017 Pediatric Rheumatology Symposium

    The myositis-specific autoantibody and myositis-associated autoantibody phenotypes in Japanese juvenile idiopathic inflammatory myopathies

    Takako Miyamae, Takayuki Kishi and Hisashi Yamanaka, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan

    Background/Purpose: Demographics,clinical features,and outcomes among myositis-specific autoantibody (MSA) and myositis-associated autoantibodies (MAAs) subgroups were assessed in children with juvenile idiopathic inflammatory myopathies (JIIM). Methods: MSAs…
  • Abstract Number: 85 • 2017 Pediatric Rheumatology Symposium

    Characteristics of anti-MDA5 autoantibody-associated Juvenile Dermatomyositis (JDM) in North America

    Gulnara Mamyrova1, Takayuki Kishi2, Ira N Targoff3, Rodolfo V Curiel1, Frederick W Miller2, Lisa G Rider1,2 and The Childhood Myositis Heterogeneity Collaborative Study Group, 1Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC, 2Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institute of Health, Bethesda, MD, 3VA Medical Center, University of Oklahoma Health Sciences Center, and Oklahoma Medical Research Foundation, Oklahoma City, OK

    Background/Purpose: Anti-MDA5 Abs have been reported to associate with clinically amyopathic and classic dermatomyositis (DM), with severe progressive interstitial lung disease (ILD) and poor prognosis…
  • Abstract Number: 3203 • 2016 ACR/ARHP Annual Meeting

    Analysis of Efficacy and Safety of Cyclophosphamide in Juvenile Dermatomyositis Using a Large National UK Cohort

    Claire Deakin1, Raquel Campanilho-Marques2, Stefania Simou3, Elena Moraitis4, Eleanor Pullenayegum5,6, Lucy R Wedderburn4,7,8, Clarissa Pilkington9 and Juvenile Dermatomyositis Research Group (JDRG), 1Infection, Inflammation and Rheumatology Section,, UCL Institute of Child Health, London, United Kingdom, 2Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, Portugal, 3Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, 4Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, United Kingdom, 5Department of Clinical Epidemiology & Biostatistics, McMaster University, Hamilton, ON, Canada, 6Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, ON, Canada, 7Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 8Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 9Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

    Background/Purpose:  Cyclophosphamide (CYC) has been used as a second-line agent in the treatment of severe or refractory JDM. The published literature on the efficacy of…
  • Abstract Number: 1365 • 2016 ACR/ARHP Annual Meeting

    Trends in Medication Usage in Patients with Juvenile Dermatomyositis

    Takayuki Kishi1, Nastaran Bayat2, Michael Ward3, Adam Huber4, Lan Wu1, Gulnara Mamyrova5, Ira Targoff6, William Warren-Hicks7, Frederick W. Miller2, Lisa G. Rider8 and the Childhood Myositis Heterogeneity Study Group, 1Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, NIH, Bethesda, MD, 2Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, 3National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 4IWK Health Centre, Halifax, NS, Canada, 5Department of Medicine, Division of Rheumatology, The George Washington University, Washington, DC, 6VA Medical Center, University of Oklahoma Health Sciences Center, Oklahoma Medical Research Foundation, Oklahoma City, OK, 7Social and Scientific Systems, Inc., Durham, NC, 8Environmental Autoimmunity Grp, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

    Background/Purpose:  Juvenile dermatomyositis (JDM) is a systemic autoimmune disease with characteristic rashes and chronic muscle inflammation. Because of its rarity, most therapeutic choices are based…
  • Abstract Number: 1367 • 2016 ACR/ARHP Annual Meeting

    Long-Term Outcomes and Their Predictors in Patients with Juvenile Idiopathic Inflammatory Myopathies of Adult Age: A Referral Population Study

    Sam Serafi1, Vladislav Tsaltskan2, Anna Yakovleva3, Heidi Sami1, Frederick W. Miller4, Rodolfo Curiel1, Olcay Y. Jones1,5 and Lisa G. Rider1,4, 1Rheumatology, George Washington University, Washington, DC, 2Internal Medicine, George Washington University, Washington, DC, 3Department of Microbiology, Immunology, and Tropical Medicine., George Washington University, Washington, DC, 4Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, NIH, Bethesda, MD, 5Pediatrics, Walter Reed National Military Medical Center, Bethesda, MD

    Background/Purpose:  To investigate the long-term outcomes and prognostic factors for pts with juvenile-onset idiopathic inflammatory myopathies (JIIM) who are currently adults. Methods: Adults with JIIM…
  • Abstract Number: 1371 • 2016 ACR/ARHP Annual Meeting

    Comparison of Patient and Physician Reported Global Disease Activity Measures in Juvenile Dermatomyositis

    Heather Tory1, David Zurakowski2, Susan Kim3 and CARRA JDM Quality Measures Workgroup, 1Rheumatology, Connecticut Children's Medical Center, Hartford, CT, 2Departments of Anesthesia and Surgery, Boston Children’s Hospital, Boston, MA, 3Division of Immunology, Boston Children's Hospital, Boston, MA

    Background/Purpose: Patient reported outcomes (PROs) are becoming increasingly recognized as important in the care of patients with chronic diseases, such as Juvenile Dermatomyositis (JDM); however,…
  • Abstract Number: 1372 • 2016 ACR/ARHP Annual Meeting

    Comparison of the Printo 2010 and Printo/International Myositis and Clinical Studies Group (IMACS) 2016 Improvement Criteria in the Printo Trial in New Onset Juvenile Dermatomyositis

    Gabriella Giancane1, Claudio Lavarello1, Angela Pistorio1, Lisa G. Rider1, Rohit Aggarwal1, Sheila Oliveira1, Rubén J. Cuttica1, Michel Fischbach2, Gary Sterba1, Karine Brochard2, Frank Dressler1, Patrizia Barone1, Rubén Burgos-Vargas1, Elizabeth C. Chalom1, Marine Desjonqueres1, Graciela Espada1, Anders Fasth1, Stella M. Garay1, Rose-Marie Herbigneaux1, Claire Hoyoux1, Chantal Job-deslandre1, Frederick W. Miller1, Jiri Vencovsky1, Angelo Ravelli3, Alberto Martini3 and Nicolino Ruperto4, 1Pediatria II, Reumatologia, PRINTO, Istituto Giannina Gaslini, Genoa, Italy, 2Pediatria II, Reumatologia, PRINTO, Istituto Giannina Gaslini, GENOA, Italy, 3Istituto Giannina Gaslini, Genoa, Italy, 4Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy

      Background/Purpose:  Juvenile dermatomyositis (JDM) is a systemic autoimmune disease characterized by chronic skeletal muscle inflammation with weakness and skin involvement. The Paediatric Rheumatology INternational…
  • Abstract Number: 2424 • 2016 ACR/ARHP Annual Meeting

    Novel Autoantigens for Anti- Endothelial Cell Antibodies in Pediatric Rheumatic Diseases Identified By Proteomics

    Rie Karasawa1, Mayumi Tamaki1, Toshiko Sato1, Kazuo Yudoh2 and James Jarvis3, 1Institute of Medical Science, St. Marianna University School of Medicine, Kawasaki, Japan, 2Institute of Medical Science, St. Marianna University School of Medicine, Kanagawa, Japan, 3Pediatrics, The University at Buffalo, Buffalo, NY

    Background/Purpose:  Juvenile dermatomyositis (JDM), a systemic autoimmune vasculopathy, and juvenile idiopathic arthritis (JIA) are representative rheumatic diseases in children. However the pathogenesis of these diseases…
  • Abstract Number: 2426 • 2016 ACR/ARHP Annual Meeting

    Transcriptomic Analysis of Immune Subsets in Juvenile Dermatomyositis before and after Treatment Identifies Novel Pathways Involved in Pathogenesis

    Claire Deakin1, Georg Otto2,3, Meredyth Wilkinson4, Stefanie Dowle2,3, Stefania Simou5, Lucy Marshall6, Elizabeth Rosser6, Daniel Kelberman2,3, Lucy R Wedderburn6,7,8 and Juvenile Dermatomyositis Research Group (JDRG), 1Infection, Inflammation and Rheumatology Section,, UCL Institute of Child Health, London, United Kingdom, 2Genetics & Genomic Medicine Programme, UCL Institute of Child Health, London, United Kingdom, 3National Institute for Health Research Biomedical Research Centre at Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 4Division of Medicine, University College London, London, United Kingdom, 5Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, 6Infection, Inflammation and Rheumatology Section, UCL Institute of Child Health, London, United Kingdom, 7Arthritis Research UK Centre for Adolescent Rheumatology, University College London, London, United Kingdom, 8Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

    Background/Purpose:  Although proximal muscle weakness and skin rash are the typical features of juvenile dermatomyositis (JDM), little is known about disease pathogenesis, why other features…
  • Abstract Number: 411 • 2015 ACR/ARHP Annual Meeting

    Patient Reported Outcomes in Juvenile Dermatomyositis: Assessing the Importance of Different Measures to Patients and Families

    Heather Tory1, Ruy Carrasco2, Thomas Griffin3, Melissa M. Hazen4, Adam M. Huber5, Philip Kahn6, Angela Byun Robinson7, Susan Kim8 and The Childhood Arthritis and Rheumatology Research Alliance Juvenile Dermatomyositis Quality Measures Workgroup, 1Rheumatology, Connecticut Children's Medical Center, Hartford, CT, 2Pediatric Rheumatology, Specially For Children, Austin, TX, 3Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4Rheumatology, Children's Hospital Boston, Boston, MA, 5Pediatric rheumatology, IWK Health Centre, Halifax, NS, Canada, 6Pediatric Rheumatology, New York University, New York, NY, 7Pediatrics, Rainbow Babies & Children's Hospital / Case Medical Center, Cleveland, OH, 8Division of Immunology, Boston Children's Hospital, Boston, MA

    Background/Purpose: Patient reported outcomes (PROs) are becoming increasingly important in the care of patients with chronic disease. Involving patients in their own care significantly improves…
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