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Abstracts tagged "Myopathies"

  • Abstract Number: 2670 • ACR Convergence 2024

    Sera from Patients with Idiopathic Inflammatory Myopathy Induces Muscle Weakness, Mitochondrial Dysfunction and Induction of Cytokines in Isolated Skeletal Muscle

    Cecilia Leijding1, Suchada Kaewin2, Kristofer Andreasson1, Tomas Schiffer1, Angeles Galindo-Feria3, Begum Horuluoglu1, Mattias Carlstrom1, Stefano Gastaldello1, Helene Alexanderson4, Ingrid Lundberg5 and Daniel Andersson6, 1Karolinska Institutet, Stockholm, Sweden, 2Karolinska Institutet, Stockholm, 3Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, 4Karolinska University Hospital, Stockholm, Sweden, 5Karolinska Universitetssjukhuset, Karolinska Institutet, Stockholm, Sweden, 6Karolinska Institutet, Solna, Sweden

    Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune inflammatory muscle disorders characterized by symmetrical skeletal muscle weakness and accelerated fatigue. Although signs…
  • Abstract Number: 1139 • ACR Convergence 2024

    Comprehensive Single-cell Profiling of Diverse Circulating Immune Cells in Idiopathic Inflammatory Myopathies Identifies a Novel Pathogenic Subset of Monocytes

    Shinji Izuka1, Toshihiko Komai2, Hayato Yuuki2, Ikuko Ueda3, Manabu Fujimoto4, Hiroyuki Fukui5, Masaru Takeshita6, Natsuka Umezawa7, Shinsuke Yasuda7, Mitsutaka Yasuda8, Yuichiro Fujieda9, Tatsuya Atsumi9, Takeshi Iwasaki10, Akio Morinobu10, Yuya Kondo11, Isao Matsumoto11, Toshio Kawamoto12, Masakazu Matsushita12, Naoto Tamura13, Taro Iwamoto14, Hiroshi Nakajima14, Ken Yoshida15, Takeo Isozaki16, Nobuyuki Yajima16, Keiichi Sakurai17, Kimito Kawahata17, Yasuyuki Kamata18, Kojiro Sato18, Yoshiya Tanaka19, Akari Suzuki20, Kazuhiko Yamamoto21, Tomohisa Okamura22 and Keishi Fujio2, 1Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Tokyo, Japan, 2Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Bunkyo, Tokyo, Japan, 3Department of Dermatology, Osaka University Graduate School of Medicine, Suita, Japan., Suita, Japan, 4Department of Dermatology, Osaka University Graduate School of Medicine, Suita, Japan., Suita, Osaka, Japan, 5Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan., Tokyo, Japan, 6Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan, 7Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan., Tokyo, Japan, 8Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan., Sapporo, Japan, 9Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan, Sapporo, Japan, 10Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan., Kyoto, Japan, 11Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan., Tsukuba, Japan, 12Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan., Tokyo, Japan, 13Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan, 14Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan., Chiba, Japan, 15Division of Rheumatology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan., Tokyo, Japan, 16Division of Rheumatology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan., Tokyo, Japan, 17Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan., Kawasaki, Japan, 18Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Tochigi, Japan., Tochigi, Japan, 19Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Fukuoka, Japan, 20Laboratory for Autoimmune Diseases, RIKEN Center for Integrative Medical Sciences, Yokohama, Kanagawa, Japan., Kanagawa, Japan, 21Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Laboratory for Autoimmune Diseases, RIKEN Center for Integrative Medical Sciences, Yokohama, Kanagawa, Japan., Kawasaki, Japan, 22Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan., Department of Functional Genomics and Immunological Diseases, Graduate School of Medicine, The University of Tokyo, Japan., Bunkyo, Tokyo, Japan

    Background/Purpose: Idiopathic inflammatory myopathies (IIMs) are heterogeneous diseases, making it crucial to identify distinct pathological processes to improve a treatment strategy. Transcriptomic analyses have revealed…
  • Abstract Number: 1965 • ACR Convergence 2024

    Artificial Intelligence Assisted Interpretation of Lung Ultrasound Imaging for the Detection of Interstitial Lung Disease

    Robert Fairchild1, Diane Mar2, Mariani Deluna3, Matthew Baker4, suzanne Tamang5, Henry Guo3, David Fiorentino4 and Lorinda Chung6, 1Stanford University, San Francisco, CA, 2VA Palo Alto / Stanford, Palo Alto, CA, 3Stanford University, Palo Alto, CA, 4Stanford University, Menlo Park, CA, 5Stanford University, Atherton, CA, 6Stanford University, Woodside, CA

    Background/Purpose: Interstitial lung disease (ILD) is a serious complication of SSc and inflammatory myopathy (IM), necessitating accurate and early detection for improved outcomes.  Lung ultrasound…
  • Abstract Number: 1168 • ACR Convergence 2023

    Evaluation of Agreement Between Functional and Radiological Changes in Idiopathic Inflammatory Myositis (IIM) Associated Interstitial Lung Disease (ILD)

    Sonali Narain1, Can Hu2, Michael Liu1, Himanshu Vashistha1, Kourosh Shargani3 and Galina Marder1, 1Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, 2Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Fort Lee, NJ, 3Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Manhasset, NY

    Background/Purpose: ILD is a major cause of morbidity and mortality in IIM. The diagnosis and progression of ILD often relies on high resolution computed tomography…
  • Abstract Number: 1971 • ACR Convergence 2023

    Muscle Pathology in Patients with Primary Biliary Cholangitis

    Jose Cesar Milisenda1, Iago Pinal-Fernandez2, Katherine Pak2, Maria Casal-Dominguez3, Yaiza Duque-Jaimez4, Gloria Garrabou1, Sandra Muñoz-Braceras5, Mariona Guitart-Manpel1, Jose Jiram Torres-Ruiz5, Ester Tobias4, Maria Dolores Cano4, Iban Aldecoa4, Josep Maria Grau1 and Andrew Mammen3, 1Muscle Research Unit, Internal Medicine Service, Hospital Clinic, Barcelona, Spain, 2National Institutes of Health, Bethesda, MD, 3NIH, Bethesda, MD, 4Hospital Clínic de Barcelona, Barcelona, Spain, 5Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD

    Background/Purpose: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease that primarily affects middle-aged women. It is characterized by elevated serum alkaline phosphatase levels,…
  • Abstract Number: 0169 • ACR Convergence 2023

    Added Value of Anti-HMGCR and Anti-SRP Antibodies in the Diagnosis of Immune Mediated Necrotizing Myopathy: An Outcome and Cost Comparison from the USA Perspective

    Alejandro Figueroa-Lara1, Mary Ann Aure2 and Carmen Andalucia1, 1Werfen, San Diego, CA, 2Werfen, Chula Vista, CA

    Background/Purpose: Immune-mediated necrotizing myopathies (IMNM) is a subgroup of immune-mediated myopathies (IMM). The diagnosis of IMNM relied on the presence of proximal muscle weakness, high…
  • Abstract Number: 1172 • ACR Convergence 2023

    Relapse Rate After Glucocorticoid-free Remission in Idiopathic Inflammatory Myopathies with Validation of the International Myositis Assessment & Clinical Studies Group (IMACS) Criteria for Remission and Relapse

    Hideaki Tsuji1, Fabricio Espinosa-Ortega2, Maryam Dastmalchi2, Ingrid Lundberg2 and Karin Lodin3, 1Division of Rheumatology, Department of Medicine, Karolinska Institutet; Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, 2Division of Rheumatology, Department of Medicine, Karolinska Institutet; Department of Gastroenterology, Dermatotology, Rhematology, Karolinska Universitetssjukhuset, Stockholm, Sweden, 3Division of Rheumatology, Department of Medicine, Karolinska Institutet; Department of Gastroenterology, Dermatotology, Rhematology, Karolinska Universitetssjukhuset, Huddinge, Sweden

    Background/Purpose: Our aim was to explore whether maintenance of remission in patients with idiopathic inflammatory myopathy (IIM) depends on glucocorticoids (GCs) after achieving remission. Therefor…
  • Abstract Number: 2461 • ACR Convergence 2023

    Transcriptional Derepression of CHD4/NuRD-regulated Genes in the Muscle of Patients with Dermatomyositis and anti-Mi2 Autoantibodies

    Iago Pinal-Fernandez1, Jose Cesar Milisenda2, Katherine Pak1, Sandra Muñoz-Braceras3, Maria Casal-Dominguez3, Jose Jiram Torres-Ruiz3, Stefania Dell´Orso4, Faiza Naz4, Gustavo Gutierrez-Cruz4, Yaiza Duque-Jaimez2, Ana Matas-Garcia2, Joan Padrosa5, Francesc J Garcia-Garcia2, Mariona Guitart-Manpel2, Gloria Garrabou2, Ernesto Trallero-Araguas6, Brian Wallit7, Julie Paik8, Jemima Albayda8, Lisa Christopher-Stine8, Tom Lloyd9, Josep Maria Grau2, Albert Selva-O’Callaghan6 and Andrew Mammen10, 1National Institutes of Health, Bethesda, MD, 2Muscle Research Unit, Internal Medicine Service, Hospital Clinic, Barcelona, Spain, 3Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 4National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 5CIBERER, Barcelona, Spain, Barcelona, Spain, 6Systemic Autoimmune Disease Unit, Vall d’Hebron Institute of Research, Barcelona, Spain, 7National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD, 8Johns Hopkins University, Baltimore, MD, 9Johns Hopkins University School of Medicine, Baltimore, MD, 10NIH, Bethesda, MD

    Background/Purpose: Myositis is a heterogeneous family of diseases including dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome (AS), and inclusion body myositis (IBM). Myositis-specific autoantibodies…
  • Abstract Number: 0290 • ACR Convergence 2023

    Patient-reported Quality of Life and Working Status Outcomes in Ambulatory Patients with Idiopathic Inflammatory Myopathy

    Zhao Peng1, Yanhong Wang2, Ning Liu3, Shuang Zhou4, Jiuliang zhao1, Dong Xu5, Mengtao Li6, chanyuan wu4 and Qian Wang4, 1Peking Union Medical College, Beijing, China, 2Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China, 3Division of Rheumatology, Department of Medicine, The University of Hong Kong-Shenzhen Hospital, HongKong, China, 4Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China, 5Department of Rheumatology, Peking Union Medical College, Beijing, China, 6Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    Background/Purpose: To investigate the health-related quality of life (HR-QoL), work productivity and activity impairment, and associated factors among patients with idiopathic inflammatory myopathy (IIM). Methods:…
  • Abstract Number: 1174 • ACR Convergence 2023

    Anti-HMGCR Immune-mediated Necrotising Myopathy: Calculation of Incidence and Confirmation of Low Malignancy Risk in Two Independent Cohorts. a Retrospective Case Review

    Thomas Khoo1, Xia Lyu2, James Lilleker1, Janine Lamb1, Vidya Limaye3 and Hector Chinoy4, 1University of Manchester, Manchester, United Kingdom, 2Department of Rheumatology, Shanghai Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China, 3Royal Adelaide Hospital, Adelaide, Australia, 4The University of Manchester, Sale, United Kingdom

    Background/Purpose: Immune-mediated necrotising myopathy (IMNM) is a rare complication of statin therapy, associated with the development of anti-HMGCR antibodies directed against the enzymatic target of…
  • Abstract Number: 2462 • ACR Convergence 2023

    Mitochondrial-Mediated Neutrophil Activation in Dermatomyositis (DM) and Inclusion Body Myositis (IBM): Insights into Pathogenesis and Therapeutic Implications

    Jorge Armando Gonzalez-Chapa1, Jemima Albayda2, Begum Horuluoglu3, Despina Michailidou1, Marina Barguil Macedo1, Lisa Christopher-Stine2, Ingrid Lundberg4 and Christian Lood1, 1University of Washington, Seattle, WA, 2Johns Hopkins University, Baltimore, MD, 3Karolinska Institutet, Stockholm, Sweden, 4Division of Rheumatology, Department of Medicine, Karolinska Institutet; Department of Gastroenterology, Dermatotology, Rhematology, Karolinska Universitetssjukhuset, Stockholm, Sweden

    Background/Purpose: Dermatomyositis (DM) and inclusion body myositis (IBM) are characterized by muscle weakness and inflammation, with emerging evidence of mitochondrial and neutrophil involvement. Prior work…
  • Abstract Number: 0291 • ACR Convergence 2023

    Myopathy Related to Small-to-medium-sized Vessel Vasculitis: Immunopathological Characteristics

    Shun Nomura, Yasuhiro Shimojima, Takanori Ichikawa, Daigo Miyazaki, Dai Kishida and Yoshiki Sekijima, Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan

    Background/Purpose: Muscular involvement develops as the initial manifestation of small-sized vessel vasculitis (SV) and medium-sized vessel vasculitis (MV).The musculoskeletal lesion has been found as a…
  • Abstract Number: 1175 • ACR Convergence 2023

    Inclusion of All Myositis Specific Autoantibodies or Other Rashes Leads to Better Sensitivity but Lower Specificity of 2017 EULAR/ACR Myositis Classification Criteria for Dermatomyositis

    Yusra Hasan1, Katharine Ching Chung2, Dana Ascherman3, Siamak Moghadam-Kia4, Chester V. Oddis3 and Rohit Aggarwal3, 1CHI Health Creighton University Medical Center, Omaha, NE, 2University of Pittsburgh Medical Center, Mckeesport, PA, 3University of Pittsburgh, Pittsburgh, PA, 4University of Pittsburgh Medical Center, Pittsburgh, PA

    Background/Purpose: Idiopathic Inflammatory Myopathies (IIM), collectively known as myositis, are heterogeneous disorders characterized by muscle weakness and muscle inflammation. ACR/EULAR classification criterion for IIM was…
  • Abstract Number: 2463 • ACR Convergence 2023

    Efficacy and Safety of Car-T-Cell Treatment in Refractory Antisynthetase Syndrome – Data of the First Three Patients

    Jule Taubmann1, Johannes Knitza2, Fabian Müller3, Sebastian Boeltz1, Simon Voelkl4, Michael Aigner4, Arnd Kleyer5, Ioanna Minnopoulou1, Regina Gary4, Sascha kretschmann4, Andreas Mackensen4 and Georg Schett6, 1Department of Internal Medicine 3, Rheumatology and Immunology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, 2Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, 3Friedrich Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 4Department of Internal Medicine 5, Hematology and Oncology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, 5University Hospital Erlangen, Erlangen, Germany, 6Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany

    Background/Purpose: Antisynthetase syndrome (ASS) can be very severe affecting the lungs, the skin and the joints next to the muscles. ASS can take a refractory…
  • Abstract Number: 0295 • ACR Convergence 2023

    Pneumocystis Jirovecii Pneumonia in Patients with Inflammatory Myopathies

    Cody Bruggemeyer1, Sanket Shah2, Didem Saygin3 and Mike Putman4, 1Medical College of Wisconsin, Wauwatosa, WI, 2Medical College of Wisconsin, Milwaukee, WI, 3University of Pittsburgh, Pittsburgh, PA, 4Division of Rheumatology, Medical College of Wisconsin, Milwaukee, WI

    Background/Purpose: Pneumocystis Jirovecii Pneumonia (PJP) is an opportunistic fungal infection with high morbidity and mortality rates. Few studies to date have assessed the incidence of…
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