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Abstracts tagged "dermatomyositis"

  • Abstract Number: 0176 • ACR Convergence 2022

    Diagnostic Accuracy and Clinical Relevance of Muscle Biopsy in Patients with Suspected Myositis

    Kastriot Kastrati1, Nasim NAKHOST LOTFI2, Marwa Tawfik3, Ellen Gelpi2, Romana Hoeftberger2, Hans-Peter Kiener2, Daniel Aletaha4, Michael Bonelli5 and Helga Radner5, 1Division of Rheumatology, Vienna, Austria, 2Medical University Vienna, Vienna, Austria, 3Suez Canal University, Ismailia, Egypt, 4Medical University Vienna, Wien, Austria, 5Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria

    Background/Purpose: Diagnosis of idiopathic inflammatory myopathies (IIM) represents a clinical challenge and muscle biopsy is regarded as a fundamental procedure for confirming diagnosis. However, the…
  • 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: 0707 • ACR Convergence 2021

    Association of Pneumomediastinum with Poor Prognosis in Patients with Myositis-Associated Interstitial Lung Disease

    Kazuki Hirano1, Azusa Kojima1, Akira Ishii1, Mai Sugiyama1, Yuto Izumi1, Noriko Sasaki2, Yuji Hosono1, Chiho Yamada1 and Shinji Sato3, 1Tokai University School of Medicine, Isehara, Japan, 2tokai university, sagamihara-city, Japan, 3Tokai University, Isehara, Japan

    Background/Purpose: Pneumomediastinum is an important complication in patients with myositis-associated interstitial lung disease (ILD). Patients with myositis and ILD who had pneumomediastinum during the disease…
  • Abstract Number: 0709 • ACR Convergence 2021

    Lower HDL-associated Apolipoprotein A-I Levels Associate with Presence of Calcinosis in Adult Dermatomyositis

    Sangmee Bae1, Ani Shahbazian2, Jennifer Wang2 and Christina Charles-Schoeman2, 1University of California Los Angeles, Los Angeles, CA, 2Department of Medicine, University of California, Los Angeles, Los Angeles, CA

    Background/Purpose: Calcinosis is a sequelae of juvenile and adult dermatomyositis (DM) associated with significant morbidity and poor quality of life. It is hypothesized that inflammatory…
  • Abstract Number: 0444 • ACR Convergence 2021

    COVID-19 Vaccination in Autoimmune Disease (CoVAD) Study: Interim Analysis of Safety in Idiopathic Inflammatory Myopathies from a Large Multicentre Global Survey

    Latika Gupta1, James Lilleker2, Vishwesh Agarwal3, Sinan Kardes4, Marcin Milchert5, Tamer Gheita6, Babur Salim7, Tsvetelina Velikova8, Oliver Distler9, Hector Chinoy10, Vikas Aggarwal1 and Rohit Aggarwal11, 1Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India, 2Division of Musculoskeletal and Dermatological Sciences, School of Biological Sciences, The University of Manchester, Centre for Musculoskeletal Research,, Manchester, United Kingdom, 3Mahatma Gandhi Mission Medical College, Navi Mumbai, India, 4Medical Ecology and Hydroclimatology Istanbul Faculty of Medicine, Istanbul, Turkey, 5Katedra Reumatologii i Chorób Wewnętrznych, Klinika Chorób Wewnętrznych Reumatologii Diabetologii Geriatrii i Immunologii Klinicznej PUM, ul. Unii Lubelskiej 1, 71-252 Szczecin,, Szczecin, Poland, 6Cairo University, Egypt, Cairo, Egypt, 7Fauji Foundation Hospital, Rawalpindi, Pakistan, 8University Hospital Lozenetz, Sofia, Bulgaria, 9Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, 10University of Manchester, Salford, United Kingdom, 11University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Numerous Covid-19 vaccines have demonstrated efficacy and safety against SARS-CoV-2 in general population. Despite favorable adverse effect (ADRs) profile of vaccines, there have been…
  • Abstract Number: 0710 • ACR Convergence 2021

    Patients with Recent Onset, Active Polymyositis (PM), Dermatomyositis (DM) and Antisynthetase Syndrome (ASS) Have Reduced Muscle Endurance but Not Reduced Muscle Strength Compared to Patients with Established, Low-active Disease

    Kristofer Andreasson1 and Helene Alexanderson2, 1Karolinska Institute, Stockholm, Sweden, 2Karolinska Institutet, Stockholm, Sweden

    Background/Purpose: To analyze for differences in quadriceps maximal isometric voluntary contraction (MVIC), muscle endurance (ME), muscle mass and fat mass (m. vastus lateralis, mid-thigh) in…
  • Abstract Number: 0513 • ACR Convergence 2021

    Spirulina Activates IFNγ via TLR4 in Dermatomyositis Skin and Peripheral Blood

    DeAnna Diaz1, Thomas Vazquez2, Christina Bax3, Jay Patel4, Madison Grinnell5, Emily Keyes6, Yubin Li5 and Victoria Werth7, 1Philadelphia College of Medicine, Philadelphia, PA, 2FIU Wertheim College of Medicine, Virginia Beach, VA, 3Corporal Michael J. Crescenz VAMC/ Department of Dermatology, U Penn, Philadelphia, PA, 4Corporal Michael J. Crescenz VAMC, Department of Dermatology, U Penn, Philadelphia, NJ, 5Corporal Michael J. Crescenz VAMC, Department of Dermatology, U Penn, Philadelphia, PA, 6Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania and Philadelphia VAMC, Philadelphia, PA, 7University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Our group has previously shown that Spirulina, a popular herbal supplement with purported immune boosting effects, is temporally associated with dermatomyositis (DM) onset and…
  • Abstract Number: 0713 • ACR Convergence 2021

    MDA5 Helicase Domains Identified as the Main Targets of Anti-MDA5 Autoantibodies in European Dermatomyositis Patients

    Eveline Van Gompel1, Catia Cerqueira2, Karine Chemin3, Begum Horuluoglu1, Angeles Shunashy Galindo-Feria4, khaled amara5, Edvard Wigren6, Susanne Gräslund6, Ellen De Langhe7, Olivier Benveniste8 and Ingrid E Lundberg6, 1Karolinska Institutet, Stockholm, Sweden, 24Dcell, Montreuil, France, 3Division of Rheumatology, Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden, 4Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, 5Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, 6Karolinska Institutet, Karolinska University Hospital, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden, Stockholm, Sweden, 7KU Leuven, Leuven, Belgium, 8UPMC, Paris, France

    Background/Purpose: The presence of anti-melanoma differentiation associated protein 5 (MDA5) autoantibodies in myositis patients is associated with mucocutaneous ulcerations, (rapidly progressing) interstitial lung disease (RPILD),…
  • Abstract Number: 0684 • ACR Convergence 2021

    Extended Report: Successful Treatment anti-MDA5 Antibody-positive Interstitial Lung Disease with Plasma Exchange Therapy

    Yoshiyuki Abe1, Masahiro Kogami2, Makio Kusaoi2, Kurisu Tada3, Ken yamaji4 and Naoto Tamura3, 1Juntendo University School of Medicine, Tokyo, Japan, 2Juntendo University, Tokyo, Japan, 3Department of Internal Medicine and Rheumatology, Juntendo University Faculty of Medicine, Tokyo, Japan, 4Juntendo University School of Medicine Internal Medicine and Rheumatology, Tokyo, Japan

    Background/Purpose: This study was extended report from our previous study that “Successful treatment of anti-MDA5 antibody-positive refractory interstitial lung disease with plasma exchange therapy” (Rheumatology…
  • Abstract Number: 0714 • ACR Convergence 2021

    Diagnostic Accuracy of Electromyogram for Myositis

    Lois bolko1, Sarah Leonard Louis2, Jean hugues Salmon1, Olivier Benveniste3, Yves Allenbach3 and Thierry Maisonobe4, 1Rheumatology, CHU maison blanche, Reims, Reims, France, 2Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Neuropathology, Paris, France, 3Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Clinical Immunology and Internal Medicine, Paris, France, 4Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Neurology, Paris, France

    Background/Purpose: The skeletal muscle biopsy is the gold standard for idiopathic inflammatory myopathies (IIM) diagnosis. Myositis specific antibodies are diagnostic biomarkers for IIM but are…
  • Abstract Number: 0687 • ACR Convergence 2021

    Clinical Features and Prognosis of a Large North American Cohort of Adult MDA5+ Dermatomyositis

    Christopher Mecoli1, Eleni Tiniakou2, William Kelly2, Jemima Albayda2, Julie Paik2, Brittany Adler2, Andrew Mammen3, Cheng Ting Lin1, Sonye Danoff4, Livia Casciola-Rosen5 and Lisa Christopher-Stine2, 1Johns Hopkins University School of Medicine, Baltimore, MD, 2Johns Hopkins University, Baltimore, MD, 3National Institutes of Health, Bethesda, MD, 4Johns Hopkins Medicine, Baltimore, MD, 5Johns Hopkins, Baltimore, MD

    Background/Purpose: We describe a single-center North American adult cohort of MDA5-positive DM, with emphasis on the subgroup of patients that experience drug-free long-term remission. Methods:…
  • Abstract Number: 0719 • ACR Convergence 2021

    Increased Risk of Venous Thromboembolism in Adult Patients with Idiopathic Inflammatory Myopathies

    Gözde Kübra Yardımcı1, Arzu Taghiyeva2, Enes Erul2, Bayram Farisogulları3, Levent Kilic1 and Sule Apraş Bilgen1, 1Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, 2HACETTEPE UNIVERSITY, Ankara, Turkey, 3Division of Rheumatology, Department of Internal Medicine Faculty of Medicine, Hacettepe University, Ankara, Turkey

    Background/Purpose: Idiopathic inflammatory myopathies (IIMs) patients have a higher risk of venous thromboembolism (VTE) [1]. We aimed to assess the incidence of thrombosis and associated…
  • Abstract Number: 0688 • ACR Convergence 2021

    Standardized Prevalence Ratios of Cancer in Idiopathic Inflammatory Myositis

    Christopher Mecoli1, Takeru Igusa2, Mengkun Chen3, XingYao Wang2, Jemima Albayda2, Julie Paik2, Eleni Tiniakou2, Brittany Adler2, Carrie Richardson4, William Kelly2, Sonye Danoff5, Andrew Mammen6, Elizabeth Platz3, Antony Rosen2, Lisa Christopher-Stine2, Livia Casciola-Rosen7 and Ami Shah8, 1Johns Hopkins University School of Medicine, Baltimore, MD, 2Johns Hopkins University, Baltimore, MD, 3Johns Hopkins School of Public Health, Baltimore, MD, 4Rush University Medical Center, Chicago, IL, 5Johns Hopkins Medicine, Baltimore, MD, 6National Institutes of Health, Bethesda, MD, 7Johns Hopkins, Baltimore, MD, 8Johns Hopkins Rheumatology, Baltimore, MD

    Background/Purpose: Whether the likelihood of a cancer diagnosis in idiopathic inflammatory myopathy (IIM) patients differs by autoantibody type is not fully characterized. To inform cancer…
  • Abstract Number: 0720 • ACR Convergence 2021

    The Clinical Significance of anti-PC4 and SFRS1 Interacting Protein 1 Antibody in Polymyositis and Dermatomyositis Patients

    Yuji Hosono1, Azusa Kojima1, Akira Ishii1, Yuto Izumi1, Kazuki Hirano1, Noriko Sasaki2, Chiho Yamada1 and Shinji Sato3, 1Tokai University School of Medicine, Isehara, Japan, 2tokai university, sagamihara-city, Japan, 3Tokai University, Isehara, Japan

    Background/Purpose: Many kinds of myositis specific autoantibodies are detected in sera from polymyositis (PM) and dermatomyositis (DM). Screening for autoantibodies is essential in the diagnostic…
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