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Abstracts tagged "juvenile dermatomyositis"

  • Abstract Number: 2241 • 2019 ACR/ARP Annual Meeting

    Parent Perspectives on Addressing Emotional Health for Youth with Juvenile Myositis: A Qualitative Focus Group Study

    Kaveh Ardalan1, Oluwatosin Adeyemi 2, Dawn Wahezi 3, Anne Caliendo 4, Megan L. Curran 5, Jessica Neely 6, Susan Kim 6, Colleen Correll 7, Emily Brunner 8 and Andrea Knight 9, 1Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, 2Children's Hospital of Philadelphia, Philadelphia, 3Children’s Hospital at Montefiore, New York, 4Northwestern University Feinberg School of Medicine, Chicago, 5University of Colorado, Aurora, CO, 6University of California, San Francisco, San Francisco, CA, 7University of Minnesota, Minneapolis, 8Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, 9Division of Rheumatology, The Hospital for Sick Children, Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Background/Purpose: While juvenile myositis (JM) can negatively affect quality of life, studies of the emotional health needs of youth with JM are limited. We examined…
  • Abstract Number: 2727 • 2019 ACR/ARP Annual Meeting

    Mitochondrial Contribution to Juvenile Dermatomyositis Pathogenesis

    Bhargavi Duvvuri1, Lauren Pachman 2, Richard Moore 1, Gabrielle Morgan 2, Marisa Klein-Gitelman 2, Megan L. Curran 3, Stephen Doty 4 and Christian Lood 1, 1University of Washington, Seattle, 2Northwestern University, Chicago, 3University of Colorado, Aurora, CO, 4Hospital for Special Surgery, New York

    Background/Purpose: Though mainly found intracellularly, we recently observed mitochondrial extrusion upon cell death, contributing to inflammation and organ damage in lupus-prone mice. Of note, mitochondria…
  • Abstract Number: 93 • 2019 ACR/ARP Annual Meeting

    Interferon Pathway Activation in T Follicular Helper (Tfh) Cell Subsets in Human Myositis

    Amrutesh Puranik1, Mark Jensen 2, Regine Tipon 3, Yogita Ghodke-Puranik 3, Valeria Mezzano 4, Shanmugapriya Selvaraj 5, Theresa Wampler Muskardin 4, Cynthia Loomis 4, Ann Reed 6, Lauren Pachman 7 and Timothy Niewold 5, 1NYU Langone Health, New York, NY, 2Colton Center for Autoimmunity, NYU School of Medicine, New York, NY, 3Colton Center for Autoimmunity, NYU School of Medicine, New York, 4New York University Langone Health, New York, 5New York University Langone Health, New York, NY, 6Duke University School of Medicine, Durham, NC, 7Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL

    Background/Purpose: T and B cells come together in ectopic lymphoid aggregates in myositis, suggesting that local T:B cell interactions could play a role in disease. …
  • Abstract Number: 390 • 2019 ACR/ARP Annual Meeting

    Preliminary Response to Janus Kinase (JAK) Inhibition with Baricitinib in Refractory Juvenile Dermatomyositis

    Hanna Kim1, Samantha Dill 2, Michelle O'Brien 2, Minal Jain 3, Shajia Lu 4, Wanxia Tsai 4, Yinghui Shi 5, Laura Vian 4, Massimo Gadina 4, Michelle Millwood 2, April Brundidge 2, Lisa G. Rider 6 and Robert Colbert 1, 1Pediatric Clinical Trials Unit, Pediatric Translational Research Branch, NIAMS, NIH, Bethesda, MD, 2Pediatric Clinical Trials Unit (PCTU), Office of the Clinical Director, NIAMS, NIH, Bethesda, MD, 3Rehabilitation Medicine Department, Physical Therapy Section, Clinical Research Center, NIH, Bethesda, MD, 4Translational Immunology Section, NIAMS, NIH, Bethesda, MD, 5Office of Clinical Director, NIAMS, NIH, Bethesda, MD, 6Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Bethesda, MD

    Background/Purpose: Juvenile dermatomyositis (JDM) is a systemic autoimmune disease with a prominent interferon (IFN) signature. Treatment often requires prolonged high-dose steroids and other immunosuppressive medications.…
  • Abstract Number: 399 • 2019 ACR/ARP Annual Meeting

    Seasonal and Temporal Analyses of Disease Onset and Diagnosis in Myositis Autoantibody Phenotypes in Juvenile Dermatomyositis (JDM)

    Gulnara Mamyrova1, Min Shi 2, Ira N. Targoff 3, Rodolfo V. Curiel 1, Frederick W. Miller 4 and Lisa G. Rider 5, 1George Washington University, School of Medicine and Health Sciences, Washington, DC, 2Biostatistics&Computational Biology Branch, NIEHS, NIH, Research Triangle Park, NC, 3Arthritis Immunology Section, Oklahoma Medical Research Foundation, Oklahoma City, OK, 4NIEHS, NIH, Bethesda, MD, 5Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Bethesda, MD

    Background/Purpose: Published studies suggest seasonal occurrence of disease onset and disease activity in patients with adult idiopathic inflammatory myopathies (IIM).Our objective was to evaluate seasonal…
  • Abstract Number: 433 • 2019 ACR/ARP Annual Meeting

    Comparison of PROMIS Computerized Adaptive Testing-Administered Item Banks versus Fixed Short Forms in Juvenile Myositis

    Ruchi Patel 1, Valeria Esparza 2, Jin-Shei Lai 1, Elizabeth Gray 1, Rowland Chang 3, David Cella 3 and Kaveh Ardalan4, 1Northwestern University Feinberg School of Medicine, Chicago, 2University of Illinois-Chicago College of Medicine, Chicago, 3Northwestern University Feinberg School of Medicine, Chicago, IL, 4Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago

    Background/Purpose: Compared to healthy children, youth with juvenile myositis (JM) often report poorer health-related quality of life (HRQoL). Legacy HRQoL measures may underestimate the impact…
  • Abstract Number: 771 • 2019 ACR/ARP Annual Meeting

    Von Willebrand Factor Is Localized in the Extravascular Tissue of Patients with Juvenile Scleroderma

    Natalia Vasquez-Canizares1, Beamon Agarwal 2, Tamar Rubinstein 3, Dawn Wahezi 4 and Morayma Reyes Gil 5, 1Children's Hospital at Montefiore, Bronx, NY, 2GenomeRxUS LLC, Providence, PA, 3Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY, 4Children’s Hospital at Montefiore, New York, 5Montefiore Medical Center/Albert Einstein College of Medicine, Bronx

    Background/Purpose: Von Willebrand Factor (VWF) is a glycoprotein synthesized in endothelial cells and megakaryocytes that has an essential role in primary hemostasis. There is increasing…
  • Abstract Number: 788 • 2019 ACR/ARP Annual Meeting

    Type I Interferon Score and Interferon Induced Mediators CXCL10 and Neopterin Are Correlated with Disease Activity in Juvenile Dermatomyositis

    Rebecca Nicolai1, Ivan Caiello 2, Rava' Lucilla 3, Silvia Rosina 4, Francesco Licciardi 5, Luisa Bracci Laudiero 2, Angelo Ravelli 6, Fabrizio De Benedetti 7 and Gian Marco Moneta 2, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Lazio, Italy, 2Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 3Clinical Epidemiology Unit, Ospedale Pediatrico Bambino Gesù, Roma, Italy, Rome, Italy, 4Giannina Gaslini Institute, IRCCS Clinica Pediatrica e Reumatologia, Genoa, Italy, Genoa, Italy, 5Division of Pediatric Immunology and Rheumatology, Regina Margherita Children Hospital,Turin, Italy, Turin, Italy, 6IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, 7Bambino Gesù Children’s Hospital, Rome, Italy

    Background/Purpose: Interferons (IFNs) seem to play an important role in the pathogenesis of juvenile dermatomyositis (JDM). Our group previously reported that expression of both type…
  • Abstract Number: 1751 • 2019 ACR/ARP Annual Meeting

    A Systematic Literature Review of Efficacy and Safety of Biologic Agents for the Treatment of Juvenile Dermatomyositis

    edoardo marrani1, Sarah Abu Rumeileh 1, Francesca Tirelli 2, Ilaria Maccora 3 and Gabriele Simonini 4, 1Post graduate School of pediatrics, University of Florence, Firenze, Italy, 2Post Graduate School of Pediatrics, University of Florence, Florence, Italy, Firenze, Italy, 3Post Graduate School of Pediatrics, University of Florence, Florence, Italy, Florence, Toscana, Italy, 4Anna Meyer Children's Hospital, Flotence, Italy

    Background/Purpose: Juvenile dermatomyositis (JDM) is a rare, chronic autoimmune illness characterized by symmetric, proximal muscle damages and involvement of the skin. While first line treatment…
  • Abstract Number: 1868 • 2019 ACR/ARP Annual Meeting

    Clinical Correlations of Autoantibodies Against Heat Shock Cognate 71 kDa Protein in Patients with Juvenile Dermatomyositis

    Rie Karasawa1, James N. Jarvis 2, Toshiko Sato 1, Megumi Tanaka 1, Mark Hicar 2, Kazuo Yudoh 1, Terrance O’Hanlon 3, Payam Noroozi-Farhadi 3 and Lisa G. Rider 4, 1Institute of Medical Science, St. Marianna University School of Medicine, Kawasaki, Japan, 2University at Buffalo, Jacobs School of Medicine and Biomedical Sciences, Buffalo, NY, 3NIEHS, NIH, Bethesda, MD, 4Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Bethesda, MD

    Background/Purpose: Juvenile dermatomyositis (JDM) is an inflammatory myopathy characterized by prominent vascular and perivascular inflammation in affected skeletal muscles. The mechanisms of vessel injury in…
  • Abstract Number: 1871 • 2019 ACR/ARP Annual Meeting

    Myositis-Specific Antibodies and Muscle Histopathology in Juvenile Dermatomyositis: New Insights into the Mechanism of Injury

    MaiLan Nguyen1, Vy Do 1, Paul Yell 1, Chanhee Jo 2, Jie Liu 2, Tracey Wright 3 and Chunyu Cai 1, 1University of Texas Southwestern Medical Center, Dallas, TX, 2Texas Scottish Rite Hospital for Children, Dallas, TX, 3University of Texas Southwestern, Texas Scottish Rite Hospital for Children, Children's Health, Dallas, TX

    Background/Purpose: Myositis-specific antibodies (MSAs) are identified in over half of children with JDM and are associated with distinct clinical phenotypes. MSA subtype, in combination with…
  • 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…
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