ACR Meeting Abstracts

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Abstracts tagged "dermatomyositis"

  • Abstract Number: 1370 • ACR Convergence 2022

    Abatacept in the Treatment of Refractory Juvenile Dermatomyositis

    Gulnara Mamyrova1, William Nguyen2, Hassan Awal1, Derek Jones1, Alison Ehrlich3, Kathleen Brindle4, Shahriar Haji-Momenian4, Robert Sheets5, Adeline Y. Chin6, Shajia Lu7, Massimo Gadina7, Hanna Kim8, Olcay Y. Jones9, Lisa G Rider10 and Rodolfo V Curiel1, 1Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC, 2Division of Rheumatology, Department of Medicine, George Washington University, School of Medicine and Health Sciences, Washington, DC, 3Department of Dermatology, George Washington University School of Medicine and Health Sciences, Washington, DC, 4Department of Radiology, George Washington University School of Medicine and Health Sciences, Washington, DC, 5Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, CA, 6Juvenile Myositis Therapeutic and Translation Studies Unit, PTRB, NIAMS, NIH, Bethesda, MD, 7Translational Immunology Section, NIAMS, NIH, Bethesda, MD, 8Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences; Juvenile Myositis Therapeutic and Translation Studies Unit, PTRB, NIAMS, NIH, Bethesda, MD, 9Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences; Pediatric Rheumatology, Walter Reed National Military Medical Center, Bethesda, MD, 10Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD

    Background/Purpose: This open-label 24-week study was conducted to evaluate the safety and efficacy of abatacept in patients with refractory juvenile dermatomyositis (JDM).Methods: Ten patients of…
  • Abstract Number: 1868 • ACR Convergence 2022

    B Cell Count in Juvenile Dermatomyositis: A Biomarker of Disease

    Christopher Costin1, Gabrielle Morgan2, Amer Khojah3 and Lauren Pachman4, 1Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 2Ann & Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, IL, 3Umm Al-Qura University, Makkah, Saudi Arabia, 4Northwestern's Feinberg School of Medicine. Ann and Robert H. Lurie Children's Hospital of Chicago; Stanley Manne Children's Research Institute of Chicago, Lake Forest, IL

    Background/Purpose: Juvenile Dermatomyositis(JDM) is heterogenous disease with many presentations. The underlying immune pathophysiology of JDM remains complex with varying phenotypes. B Cells remain a critical…
  • Abstract Number: 0034 • ACR Convergence 2022

    Association of Anti-Tropomyosin Alpha-4 Autoantibodies with Cutaneous Manifestations in Juvenile Dermatomyositis

    Rie Karasawa1, James Jarvis2, Toshiko Sato1, Megumi Tanaka1, Terrance P O'Hanlon3, Payam Noroozi-Farhadi4, Willy A. Flegel5, Kazuo Yudoh1 and Lisa G Rider3, 1Institute of Medical Science, St. Marianna University School of Medicine, Kawasaki, Japan, 2Department of Pediatrics, University at Buffalo Clinical and Translational Research Center, Buffalo, NY, 3Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD, 4NIEHS, NIH, Garrett Park, MD, 5NIH, Garrett Park, MD

    Background/Purpose: Juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM) are among the most common forms of inflammatory autoimmune diseases in children. JDM is characterized by…
  • Abstract Number: 0177 • ACR Convergence 2022

    COVID-19 Vaccination-related Short-term Adverse Events in Patients with Idiopathic Inflammatory Myositis and Autoimmune Multimorbidity: Results from the COVID-19 Vaccination in Autoimmune Diseases Survey

    Mrinalini Dey1, Naveen R2, Elena Nikiphorou3, Parikshit Sen4, James B. Lilleker5, Vishwesh Agarwal6, Sinan Kardes7, Jessica Day8, Marcin Milchert9, Mrudula Joshi10, Tamer A Gheita11, Babur Salim12, Tsvetelina Velikova13, Abraham Edgar Gracia-Ramos14, Ioannis Parodis15, Albert Selva O’Callaghan16, Minchul Kim17, Tulika Chatterjee17, Ai Lyn Tan18, Ashima Makol19, Arvind Nune20, Lorenzo Cavagna21, Miguel Angel Saavedra Salinas22, Samuel Shinjo23, Nelly Ziade24, Johannes Knitza25, Masataka Kuwana26, Oliver Distler27, Hector Chinoy28, John Pauling29, Chris Wincup30, Vikas Agarwal2, Rohit Aggarwal31 and Latika Gupta32, 1Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom, 2Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, 3Leiden University Medical Center & King's College London, London, United Kingdom, 4Maulana Azad Medical College, New Delhi, India, 5The University of Manchester, Manchester, United Kingdom, 6Mahatma Gandhi Missions Medical College, Lucknow, India, 7Istanbul University, Istanbul, Turkey, 8Walter and Eliza Hall Institute, Melbourne, Australia, 9Pomeranian Medical University in Szczecin, Szczecin, Poland, 10Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, India, 11Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt, 12Fauji foundation hospital Rawalpindi, Rawalpindi, Pakistan, 13Sofia University St. Kliment Ohridski, Sofia, Bulgaria, 14Instituto Mexicano del Seguro Social, Ciudad de México, Mexico, 15Karolinska Institutet, Stockholm, Sweden, 16Hospital Universitari Vall d'Hebron, Barcelona, Spain, 17University of Illinois College of Medicine Peoria, Peoria, IL, 18University of Leeds, Leeds, United Kingdom, 19Mayo Clinic, Rochester, MN, Rochester, MN, 20Southport and Ormskirk Hospital NHS Trust, Southport, United Kingdom, 21Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, Pavia, Italy, 22IMSS, Ciudad de México, Mexico, 23Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, Brazil, 24Saint-Joseph University, Beirut, Lebanon, 25Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany; Deutsches Zentrum Immuntherapie, Friedrich-Alexander-UniversityErlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 26Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 27Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 28The University of Manchester, Sale, United Kingdom, 29North Bristol NHS Trust, Bristol, United Kingdom, 30Rayne Institute, University College London, London, United Kingdom, 31Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, 32Royal Wolverhampton Trust, Wolverhampton/University of Manchester, United Kingdom

    Background/Purpose: COVID-19 vaccines are safe and effective, though patients with rare rheumatic diseases like idiopathic inflammatory myositis (IIMs), and those with multiple comorbidities continue to…
  • Abstract Number: 1372 • ACR Convergence 2022

    Telemedicine Use in the Assessment of Juvenile Dermatomyositis and Polymyositis: A Survey of Caregivers and Patients

    Stacey Tarvin1, Y. Ingrid Goh2, Nicole Taylor3, Bianca Lang4, Marietta De Guzman5, Julie Fuller6, Kristin Houghton7, Susan Kim8, Vanessa Carbone9, Kathryn Cook10, Tanya Slater9, Angela Robinson11, Liza McCann12, Charalampia Papadopoulou13, Clarissa Pilkington14, Phoebe Rushe15 and Peter Blier16, 1Riley Hospital for Children at Indiana University Health, Indianapolis, IN, 2Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute, Toronto, ON, Canada, 3University of Indianapolis, Indianapolis, IN, 4Dalhousie University - Halifax, Halifax, NS, Canada, 5Baylor College of Medicine/ Texas Children's Hospital, Houston, TX, 6UT Southwestern, Frisco, TX, 7University of British Columbia - Vancouver, Vancouver, BC, Canada, 8UCSF Benioff Children's Hospital, San Francisco, CA, 9The Hospital for Sick Children, Toronto, ON, Canada, 10Akron Children's Hospital, Copley, OH, 11Cleveland Clinic, Cleveland, OH, 12Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, 13UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, 14Great Ormond Street Hospital, London, United Kingdom, 15Royal Liverpool University Hospital, Liverpool, United Kingdom, 16Retired, Amherst, MA

    Background/Purpose: Care of patients with juvenile myositis (JM) involves complex assessments performed by highly trained specialists. Restrictions surrounding the COVID-19 pandemic resulted in many patients…
  • Abstract Number: 1869 • ACR Convergence 2022

    Looking for BlyS(BAFF) in Juvenile Dermatomyositis: A New Biomarker of Disease Activity

    Christopher Costin1, Gabrielle Morgan2, Amer Khojah3 and Lauren Pachman4, 1Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 2Ann & Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, IL, 3Umm Al-Qura University, Makkah, Saudi Arabia, 4Northwestern's Feinberg School of Medicine. Ann and Robert H. Lurie Children's Hospital of Chicago; Stanley Manne Children's Research Institute of Chicago, Lake Forest, IL

    Background/Purpose: B Cell Activating Factor (BAFF) is a cytokine that drives B Cell proliferation and maturation. B Cells remain an important factor in JDM immune…
  • Abstract Number: 0040 • ACR Convergence 2022

    Plasma Proteomic Signatures in Juvenile Dermatomyositis Highlight Novel Proteins and a Protein Module That Associate with Skin and Global Disease Activity

    Marianne Kerski1, Celine Berthier1, Alex Tsoi1, Sarah Vandenbergen1, Madison McClune1, Corey Powell1, J. Michelle Kahlenberg1 and Jessica Turnier2, 1University of Michigan, Ann Arbor, MI, 2University of Michigan, Saline, MI

    Background/Purpose: Juvenile dermatomyositis (JDM) patients display heterogenous disease phenotypes and treatment response patterns. Our study used a multiplexed proteomics assay for measurement of 3072 proteins…
  • Abstract Number: 0179 • ACR Convergence 2022

    Discordant Monozygotic Twins with Juvenile Dermatomyositis: Genetic and Clinical Studies

    Lauren Pachman1, Gabrielle Morgan2, Wilfredo Marin3, Sabah Kadri3, Kai Lee Yap3 and Amer Khojah4, 1Northwestern's Feinberg School of Medicine. Ann and Robert H. Lurie Children's Hospital of Chicago; Stanley Manne Children's Research Institute of Chicago, Lake Forest, IL, 2Ann & Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine, Chicago, IL, 3Ann & Robert H. Lurie Children's Hospital, Chicago, IL, 4Umm Al-Qura University, Makkah, Saudi Arabia

    Background/Purpose: Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease characterized by proximal weakness and skin rash. Although the exact mechanism of JDM is not…
  • Abstract Number: 1375 • ACR Convergence 2022

    Short-term Efficacy of Baricitinib in Children with Refractory And/or Severe Juvenile Dermatomyositis

    zhaoling wang1, meiping lu2 and qi zheng1, 1Department of Rheumatology Immunology and Allergy, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China, 2Children’s Hospital of Zhejiang University Medical School, Zhejiang, China

    Background/Purpose: To evaluate the short-term efficacy and safety of baricitinib in children withrefractory and/or severe juvenile dermatomyositis (rsJDM) in a real-world setting. Methods: A monocentric…
  • Abstract Number: 1870 • ACR Convergence 2022

    Clinical Outcomes Following Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) Infection and Vaccination in Patients with Anti-Melanoma Differentiation Associated Gene 5 (MDA5) Antibody Positive Dermatomyositis

    Richard Seto1, Sangmee Bae1 and Christina Charles-Schoeman2, 1University of California Los Angeles, Los Angeles, CA, 2Division of Rheumatology, University of California, Los Angeles, Santa Monica, CA

    Background/Purpose: Patients with dermatomyositis (DM) and antibodies (ab) to melanoma differentiation associated gene 5 (MDA5) share similar clinical characteristics to patients infected with the severe…
  • Abstract Number: 0041 • ACR Convergence 2022

    Modeling Juvenile Dermatomyositis with Engineered Human Skeletal Muscle: Effects of Type I Interferonβ and Janus Kinase Inhibitors

    Lauren Covert1, George Truskey2 and Jeffrey Dvergsten1, 1Duke University Hospital, Durham, NC, 2Duke University, Durham, NC

    Background/Purpose: Upregulation of Type I interferons (IFN I), including IFNβ, is a hallmark of adult and juvenile dermatomyositis (JDM), but its role in pathogenesis is…
  • Abstract Number: 0208 • ACR Convergence 2022

    Cutaneous Dermatomyositis Disease Area and Severity Index Activity Score (CDASI-A) and Associated Patient-Reported Outcomes and Biomarkers in a Phase 2 Clinical Trial in Dermatomyositis (DM)

    Josh Dan1, Jay Patel2, Grant Sprow3, DeAnna Diaz4, Nilesh Kodali5, Rui Feng6, Barbara White7 and Victoria Werth8, 1Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, 2Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PN, 3Albert Einstein College of Medicine, Philadelphia, PA, 4Philadelphia College of Medicine, Philadelphia, PA, 5New Jersey Medical School, Coppell, TX, 6University of Pennsylvania, Philadelphia, 7SFJ Pharmaceuticals, Towson, MD, 8University of Pennsylvania and Corporal Michael J. Crescenz VA Medical Center, Philadelphia, PA

    Background/Purpose: Retrospective reviews of clinical databases from two sites have identified strong relationships between patient-reported outcomes and skin activity in DM, as measured by CDASI-A.…
  • Abstract Number: 1377 • ACR Convergence 2022

    Mental Health Screening in Juvenile Myositis: Preliminary Analysis of a Multicenter Pilot Study

    Kaveh Ardalan1, Lindsay Olson1, Jeffrey Dvergsten2, Ann Reed1, Alison Manning1, Gary Maslow1, Aruna Rikhi1, Brian Feldman3, ashley Danguecan4, Sarah Mossad4, Luana Flores Pereira4, Susan Shenoi5, Stacey Haynes5, Joanna Patten5 and Andrea Knight6, 1Duke University School of Medicine, Durham, NC, 2Duke University Hospital, Durham, NC, 3Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada, 4The Hospital for Sick Children, Toronto, ON, Canada, 5Seattle Children's Hospital and Research Center / University of Washington, Seattle, WA, 6The Hospital for Sick Children, Division of Rheumatology, Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Background/Purpose: Qualitative studies in juvenile myositis (JM) suggest high rates of emotional distress but the prevalence of mental health comorbidities is not well described. We…
  • Abstract Number: 1872 • ACR Convergence 2022

    Outcome of Idiopathic Inflammatory Myositis Patients Who Received Rituximab: A Single Centre Retrospective Study

    Fahidah Alenzi1, Shirish Sangle2 and Sanna Giovanni2, 1Princess Nourah bint Abdulrahman University, Riyadh, Saudi Arabia, 2Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom

    Background/Purpose: Idiopathic inflammatory myositis (IIM) includes a spectrum of a rare autoimmune disease characterized by proximal muscle weakness, variable skin manifestation and extra muscular manifestations.…
  • Abstract Number: 0043 • ACR Convergence 2022

    Non-invasive Tape Strip Gene Expression Profiling of Lesional Juvenile Dermatomyositis Skin Identifies Immunoregulatory Module That Associates with Skin, Muscle and Global Disease Activity

    Jessica Turnier1, Celine Berthier2, Madison McClune2, Sarah Vandenbergen2, Johann Gudjonsson2, Alex Tsoi2 and J. Michelle Kahlenberg2, 1University of Michigan, Saline, MI, 2University of Michigan, Ann Arbor, MI

    Background/Purpose: Skin inflammation in juvenile dermatomyositis (JDM) frequently persists even in the absence of active muscle disease. Tape stripping is a non-invasive skin sampling method…
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