Abstracts tagged "Myopathies"

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…
Abstract Number: 1771 • ACR Convergence 2023
Role of Periostin in the Pathophysiology and Accurate Diagnosis of Interstitial Lung Disease Associated with Autoimmune Diseases
Verónica Pulito-Cueto¹, David Iturbe-Fernández², Victor Mora-Cuesta², Joao Carlos Batista-Liz¹, Belén Atienza-Mateo³, María Sebastián-Mora¹, Virginia Portilla¹, Alfonso Corrales¹, Miguel A Gonzalez-Gay⁴, Jose Cifrian⁵, Ricardo Blanco¹ and Raquel López-Mejías¹, ¹Rheumatology Department, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, ²Immunopathology Group, Marqués de Valdecilla University Hospital-IDIVAL; Department of Pneumology, Marqués de Valdecilla University Hospital, Santander, Spain, ³Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ⁴IDIVAL and School of Medicine, UC, Santander; Department of Rheumatology, IIS-Fundación Jiménez Díaz, Madrid, Santander, Spain, ⁵Immunopathology Group, Marqués de Valdecilla University Hospital-IDIVAL; Department of Pneumology, Marqués de Valdecilla University Hospital; School of Medicine, Cantabria University, Santander, Spain
Background/Purpose: Periostin is an extracellular matrix protein that contributes to the development and repair of lung tissue [1]. Previous works have described periostin as a…
Abstract Number: 2575 • ACR Convergence 2023
Accurate Stratification of Cancer Risk in a Real-World Cohort Using the International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening
Alexander Oldroyd¹, Francisca Bozan¹, Xia Lyu², Patrick Gordon³, David Isenberg⁴, Neil McHugh⁵, Harsha Gunawardena⁶, Patrick Kiely⁷, Janine Lamb¹, Pedro Machado⁸, James Miller⁹, Sarah Tansley⁵ and Hector Chinoy¹⁰, ¹University of Manchester, Manchester, United Kingdom, ²Department of Rheumatology, Shanghai Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China, ³NHS, London, United Kingdom, ⁴University College London, London, United Kingdom, ⁵University of Bath, Bath, United Kingdom, ⁶North Bristol NHS Trust, Bristol, United Kingdom, ⁷St George’s University Hospitals NHS Foundation Trust, London, United Kingdom, ⁸Centre for Rheumatology & Department of Neuromuscular Diseases, University College London, London, UK. Department of Rheumatology, Northwick Park Hospital, London North West University Healthcare NHS trust, London, UK., London, United Kingdom, ⁹Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, United Kingdom, ¹⁰The University of Manchester, Sale, United Kingdom
Background/Purpose: Adult-onset idiopathic inflammatory myopathy (IIM) is associated with an increased cancer risk within three years prior to or following IIM onset. The International Guideline…
Abstract Number: 0296 • ACR Convergence 2023
Does Presence of Anti-SSa and Ro52 Influence the Progression of Interstitial Lung Disease (ILD) in Idiopathic Inflammatory Myopathies (IIM)
Sonali Narain¹, Can Hu², Michael Liu¹, 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: Anti-SSa and Ro52 are commonly present in IIM. We previously reported an association of a more severe early course of IIM-ILD with positive Ro52.…
Abstract Number: 1914 • ACR Convergence 2023
Intravenous Immunoglobulins in the Treatment of Idiopathic Inflammatory Myopathies in a Region of Northern Spain: Analysis by Subtypes
Cristina Corrales Selaya¹, David Martínez-Lopez², Diana Prieto-Peña¹, Fabricio Benavides¹, Jose Luis Martin-Varillas³ and Ricardo Blanco⁴, ¹Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²Hospital Sierrallana, Torrelavega, Spain, ³Hospital de Laredo, Laredo, Spain, ⁴Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases characterised mainly by the presence of inflammation and muscle weakness. The main subtypes of…
Abstract Number: 2577 • ACR Convergence 2023
Frequency of Atherosclerotic Cardiovascular Disease Following a Diagnosis of Idiopathic Inflammatory Myopathy: Data from a Large National Registry
Astia Allenzara¹, Carolina Alvarez², Amanda Nelson³ and Galen Foulke⁴, ¹University of North Carolina, Chapel Hill, NC, ²University of North Carolina at Chapel Hill, Miami, FL, ³University of North Carolina at Chapel Hill, Chapel Hill, NC, ⁴Penn State MS Hershey Medical Center, Hershey, PA
Background/Purpose: Idiopathic inflammatory myopathies confer an increased risk of morbidity from cardiovascular disease. Prior work has noted more than double risk of cardiovascular events for…
Abstract Number: 0297 • ACR Convergence 2023
Characteristics of Anti-MDA-5 Associated Dermatomyositis in Southern California with a Large Hispanic Population: Case Series
Eaman Alhassan¹, Belina Yi², Jack Rodman¹ and Leanna Wise³, ¹University of Southern California, Los Angeles, CA, ²Children's Hospital Los Angeles, Los Angeles, CA, ³LAC+USC/Keck Medicine of USC, Pasadena, CA
Background/Purpose: There is little to no data about the presentation and clinical course of anti-melanoma differentiation-associated gene-5 antibody (anti-MDA-5) dermatomyositis patients in a primarily U.S.…
Abstract Number: 1946 • ACR Convergence 2023
Zetomipzomib Demonstrates Favorable Long-term Safety and Tolerability Profile Without Signs of Immunosuppression: Results from the PRESIDIO Study and Its Open-label Extension Study in Patients with Dermatomyositis and Polymyositis
Rohit Aggarwal¹, Namita Goyal², Diana Lam³, Allen Ng³, Richard Leff⁴, Kathryn Ray³, Eunmi Park³ and Noreen Henig³, ¹University of Pittsburgh, Pittsburgh, PA, ²UCI Health, Orange, CA, ³Kezar Life Sciences, South San Francisco, CA, ⁴Kezar Life Sciences, Chadds Ford, PA
Background/Purpose: Polymyositis (PM) and dermatomyositis (DM) are chronic autoimmune diseases characterized by muscle inflammation and weakness. In a collagen-induced myositis mouse model, immunoproteasome inhibition has…

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