Abstracts tagged "juvenile dermatomyositis"

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: 1924 • 2019 ACR/ARP Annual Meeting
Assessing Psychosocial Needs in Juvenile Dermatomyositis Patients Across the United Kingdom
Polly Livermore¹ and Lucy Wedderburn ², ¹ICH/UCL, Arlesey, United Kingdom, ²ICH/UCL, London, United Kingdom
Background/Purpose: Juvenile Dermatomyositis (JDM) is a rare, autoimmune inflammatory condition primarily affecting the muscles and skin. With a mean age of onset of 7 years…
Abstract Number: 2106 • 2019 ACR/ARP Annual Meeting
Features of Pneumocystis Jirovecii Pneumonia in Juvenile Idiopathic Inflammatory Myopathy
Sara Sabbagh¹, Jessica Neely ², Jamie Lai ³, Anjali Sura ⁴, Kelly Rouster-Stevens ⁵, Svetlana Lvovich ⁶, Tara McGrath ⁷, Lori Tucker ⁸, Heinrike Schmeling ⁹, Jordan Roberts ¹⁰, Lisa G. Rider ¹¹ and Susan Kim ², ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, MD, ²University of California, San Francisco, San Francisco, CA, ³Department of Pediatrics, University of Colorado School of Medicine, Division of Pediatric Rheumatology, Children’s Hospital Colorado, Aurora, CO, ⁴University of Michigan, Ann Arbor, MI, ⁵Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, ⁶Section of Rheumatology, Department of Pediatrics, St. Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, PA, ⁷Division of Rheumatology, Department of Pediatrics, University of British Columbia, Vancouver, BC, Canada, ⁸British Columbia Children’s Hospital, Vancouver, Canada, ⁹Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ¹⁰Boston Children’s Hospital, Harvard Medical School, Boston, MD, ¹¹Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Bethesda, MD
Background/Purpose: Approximately 6% of adults with idiopathic inflammatory myopathy (IIM) develop Pneumocystis jirovecii pneumonia (PJP), and PJP confers higher mortality in IIM patients compared to…
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: 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…

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