Abstracts tagged "Myositis"

Abstract Number: L06 • ACR Convergence 2024
Distinct Cytokine and Cytokine Receptor Expression Patterns Characterize Different Subtypes of Inflammatory Myopathies
Raphael Kirou¹, Iago Pinal-Fernandez¹ and Andrew Mammen², ¹NIAMS/National Institutes of Health, Bethesda, MD, ²NIH, Bethesda, MD
Background/Purpose: Myositis is a term for a heterogeneous group of inflammatory myopathies, each with characteristic clinical manifestations, histopathological features, and autoantibodies, including myositis-specific autoantibodies (MSAs).…
Abstract Number: L07 • ACR Convergence 2024
The Classification Criteria for Anti-Synthetase Syndrome (Class) Project
Giovanni Zanframundo¹, Sara Faghihi Kashani², Akira Yoshida³, Eduardo Dourado⁴, Iazsmin Bauer Ventura⁵, Daphne Rivero Gallegos⁶, Aravinthan Loganathan⁷, Gianluca Sambataro⁸, Francisca Bozan⁹, Sangmee Bae¹⁰, Darosa Lim¹¹, Marta Cheli¹², Yasuhiko Yamano¹³, Francesco Bonella¹⁴, Tamera Corte¹⁵, Tracy J. Doyle¹⁶, David Fiorentino¹⁷, Miguel Gonzalez-Gay¹⁸, Marie Hudson¹⁹, Masataka Kuwana²⁰, Ingrid Lundberg²¹, Andrew Mammen²², Neil McHugh²³, Frederick Miller²⁴, Carlomaurizio Montecucco¹, Chester Oddis²⁵, Jorge Rojas-Serrano⁶, Jens Schmidt²⁶, Carlo Alberto Scirè²⁷, Albert Selva-O'Callaghan²⁸, Victoria Werth¹¹, Davide Rozza²⁹, Monica Morosini³⁰, Sara Bozzini³⁰, Lorenzo Cavagna¹, Rohit Aggarwal²⁵ and the CLASS project investigators, ¹Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, ²University of Pittsburgh School of Medicine, Pittsburgh, PA, USA and University of California, San Francisco, CA, ³Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, ⁴Unidade Local de Saùde da Regiao de Aveiro and Aveiro Rheumatology Research Centre, Aveiro, Portugal; Universidade de Lisboa, Lisboa, Portugal, ⁵University of Chicago, Chicago, IL, ⁶Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Mexico City, Mexico, ⁷Royal National Hospital for Rheumatic Diseases and University of Bath, UK; Arthritis Australia, Broadway, Glebe, NSW, Australia, ⁸University of Enna "Kore", Enna, Italy, ⁹Hospital Clinico Universidad de Chile, Santiago de Chile, Chile, ¹⁰UCLA, Los Angeles, CA, ¹¹University of Pennsylvania, Philadelphia, PA, ¹²Fondazione IRCCS Istituto Neurologico C. Besta, Milano, Italy, ¹³Tosei General Hospital, Aichi, Japan, ¹⁴University of Duisburg-Essen, Essen, Germany, ¹⁵Royal Prince Alfred Hospital and University of Sydney, Sydney, Australia, ¹⁶Brigham and Women's Hospital, Boston, MA, ¹⁷Stanford University, Redwood City, CA, ¹⁸IIS-Fundación Jiménez Díaz, Madrid, Spain, ¹⁹McGill University, Montreal, QC, Canada, ²⁰Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ²¹Karolinska Institutet, Stockholm, Sweden, ²²NIH, Bethesda, MD, ²³University of Bath, Bath, United Kingdom, ²⁴NIH, NIEHS, Durham, NC, ²⁵University of Pittsburgh, Pittsburgh, PA, ²⁶University Medical Center Goettingen, Goettingen, Germany, ²⁷Univeristy of Milano Bicocca, Milan, Italy and IRCCS San Gerardo dei Tintori Foundation, Monza, Italy, ²⁸Hospital Universitari Vall d'Hebron, Barcelona, Spain, ²⁹Italian Society for Rheumatology, Milano, Italy, ³⁰Fondazione IRCCS Policlinico San Matteo Hospital of Pavia, Pavia, Italy
Background/Purpose: The aim of this study was to develop and validate new classification criteria for Anti-Synthetase Syndrome (ASSD) using data and consensus driven methodologies and…
Abstract Number: 0005 • ACR Convergence 2024
Autoantibodies Identified in Myositis-Specific Autoantibody Negative Juvenile Myositis Patients
FIONNUALA MCMORROW¹, Lisa Rider², Neil McHugh¹ and Sarah Tansley¹, ¹University of Bath, Bath, United Kingdom, ²NIEHS, NIH, Garrett Park, MD
Background/Purpose: Myositis autoantibodies are important clinical biomarkers however, around 40% of patients with juvenile myositis (JM) are classified as ‘autoantibody negative’ (1) and therefore cannot…
Abstract Number: 0338 • ACR Convergence 2024
Anti-MDA5 Antibody-positive Dermatomyositis Associated Interstitial Lung Disease – Survival Benefit of Treatment with Rituximab, Janus Kinase Inhibitor and Glucocorticoid Combination in a Prospective Cohort
Cheryl Chun Man Ng¹, Kitty Yan Kwok¹, Chup-Hei Ho¹, Tze-Chung Ho¹, Chu-Oi Ciang² and Moon-Ho Leung¹, ¹Queen Elizabeth Hospital, Hong Kong, Hong Kong, ²QEH, Hong Kong., Hong Kong, China
Background/Purpose: Anti-melanoma differentiation-associated protein 5 antibody associated dermatomyositis (Anti-MDA5 DM) is known to be an aggressive disease. Earlier cohorts reported a 1-year mortality of 38%-57%, with rapidly…
Abstract Number: 1145 • ACR Convergence 2024
Clinico-Immunological Profile and Treatment Outcomes of Clinically Significant Diaphragmatic Weakness in Patients with Idiopathic Inflammatory Myopathies: Results from the Largest Single-Center Cohort of 16 Cases
Siddharth Jain¹, Bhargavi Pridhivi², Adil Rashid Khan², Stanzin Spalkit², Manish Soneja², Mehar Chand Sharma², Sanjeev Sinha², Uma Kumar² and Naveet Wig², ¹All India Institute of Medical Sciences, New Delhi, Delhi, Delhi, India, ²All India Institute of Medical Sciences, New Delhi, Delhi, India
Background/Purpose: Diaphragmatic myositis is rarely reported in patients with idiopathic inflammatory myopathy (IIM) but is associated with high morbidity and mortality. Epidemiology, spectrum of clinical…
Abstract Number: 1165 • ACR Convergence 2024
Dysregulation of Peripheral Blood B-cells Is Associated with Myocarditis in Patients with Idiopathic Inflammatory Myopathies
Julie Paik¹, Kevin Bermea², Carolina Duque³, Monica Mugnier³ and Luigi Adamo¹, ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins Cardiology, Baltimore, MD, ³Johns Hopkins Medicine, Baltimore, MD
Background/Purpose: Cardiac involvement, such as myocarditis, in idiopathic inflammatory myopathies (IIM) is a negative prognostic factor associated with increased mortality. The gold standard for myocarditis…
Abstract Number: 1749 • ACR Convergence 2024
Safety and Long-term Efficacy of CD19-CAR T-cell Therapy in 30 Patients with Autoimmune Disease
Melanie Hagen¹, Fabian Müller², Andreas Wirsching¹, Carlo Tur¹, Tobias Krickau³, Markus Metzler⁴, Laura Bucci¹, Christina Bergmann⁵, Janina Auth¹, Jule Taubmann⁶, Panagiotis Garantziotis⁷, Sebastian Boeltz⁸, Soraya Kharboutli⁹, Silvia Spoerl⁹, Simon Völkl¹⁰, Michael Aigner⁹, Sascha Kretschmann⁹, Ingrid Vasova⁹, Andreas MAckensen¹¹, Ricardo Grieshaber-Bouyer⁸ and Georg Schett¹², ¹Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ²Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ³Department of Pediatrics and Adolscent Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ⁴Department of Pediatrics and Adolscent Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁵Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁶Friedrich-Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁷Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ⁸Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ⁹Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ¹⁰Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ¹¹Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ¹²Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: Treating autoimmune diseases like Systemic Lupus erythematosus (SLE), Idiopathic Inflammatory Myositis (IIM) or Systemic Sclerosis (SSc) is challenging and often requires life-long immunosuppressive therapy.…
Abstract Number: 2078 • ACR Convergence 2024
Increased RAGE Expression in Myositis
Madison King¹, Rita Spathis², Elizabeth Bagley¹ and Melissa Morales³, ¹Binghamton University, Johnson City, NY, ²Binghamton University School of Pharmacy and Pharmaceutical Sciences, Johnson City, NY, ³SUNY Binghamton, Johnson City, NY
Background/Purpose: Myositis is a broad term for a group of rare autoimmune diseases that cause persistent muscle inflammation, weakness, and fatigue. Currently, first-line treatment for…
Abstract Number: 2669 • ACR Convergence 2024
Dysferlin Autoantibodies Compromise Sarcolemmal Repair Capacity and Contribute to the Pathogenesis of Idiopathic Immune Myopathies
Hannah Bulgart¹, Kassidy Banford², Shane Bruckner³, Kevin McElhanon², Nicholas Young¹, Brian Paleo², Eric Beck², Rohit Aggarwal⁴, Chester Oddis⁵, Braden Zeno⁶, Wael Jarjour⁷ and Noah Weisleder³, ¹The Ohio State University Wexner Medical Center, Columbus, OH, ²The Ohio State University, Columbus, ³Ohio State University, Columbus, OH, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁵Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ⁶Ohio State University, upper arlington, OH, ⁷The Ohio State University, Columbus, OH
Background/Purpose: Idiopathic immune myopathies (IIMs) are a group of autoimmune diseases that produce chronic inflammation and degeneration of skeletal muscle structure and function. One process…
Abstract Number: 0088 • ACR Convergence 2024
Profound B Cell Depletion and Repopulation with Predominantly Naïve B Cells in Non-Human Primates Achieved Through a Novel In Vivo CD8-Targeted Lipid Nanoparticle mRNA CAR
Aric Frantz¹, Romina Riener¹, Annabel Wang¹, Yanjie Bao¹, Yan Zhang¹, Daiki Matsuda¹, John Li¹, David Chu¹, Theresa Hunter¹, Qian Chen Yong¹, Michelle Nguyen¹, Stuart Sievers¹, Duy Nguyen¹, Scott Roberts¹, Diana Galvan¹, Jerel Boyd Vega¹, Matthew Butcher¹, Stanley Zhang¹, Stephen Flynn¹, Yi Kuo¹, Steven Tanis¹, John Scholler², Gregor Adams¹, Michael Rosenzweig¹, Priya Karmali¹, Adrian Bot¹, Carl June² and Haig Aghajanian¹, ¹Capstan Therapeutics, San Diego, CA, ²University of Pennsylvania Perelman School of Medicine, Philadelphia, PA

Background/Purpose: Ex vivo chimeric antigen receptor (CAR) T cell therapies have revolutionized cancer treatment and are demonstrating durable clinical efficacy in various autoimmune disease indications.…
Abstract Number: 0340 • ACR Convergence 2024
Progressive Pulmonary Fibrosis Predicts Mortality in Myositis-Associated Interstitial Lung Disease
Yu-Wan Liao¹, Ming-Cheng Liu¹, Pin-Kuei Fu¹ and Wen-Nan Huang², and Integrated Care Center of Interstitial Lung Disease, ¹Taichung Veterans General Hospital, Taichung, Taiwan (Republic of China), ²Taichung Veterans General Hospital, Taichung, Taiwan, Taichung, Taiwan (Republic of China)
Background/Purpose: Myositis-associated interstitial lung disease (MA-ILD) is a complex condition with a variable prognosis and diverse clinical manifestations. The 2022 ATS/ERS/JRS/ALAT criteria for PPF provide…
Abstract Number: 1146 • ACR Convergence 2024
Impact of COVID-19 on Myositis Testing Trends
Angel Kevin Garza-Elizondo¹, Rosa Icela Arvizu-Rivera², Pablo Gamez-Siller³, Daniela Alejandra Salcedo-Soto⁴, Jesus Cardenas-de la Garza⁵, Gisela Garcia-Arellano⁶, Miguel Angel Villarreal-Alarcon² and Dionicio Galarza-Delgado⁷, ¹Division of Rheumatology, University Hospital "Dr. Jose Eleuterio Gonzalez", Universidad Autonoma de Nuevo Leon, San Nicolás de los Garza, Mexico, ²Division of Rheumatology, University Hospital "Dr. Jose Eleuterio Gonzalez", Universidad Autonoma de Nuevo Leon, Monterrey, Mexico, ³Facultad de Medicina UANL, Monterrey, Nuevo León, Mexico, ⁴Hospital Universitario Dr Jose Eleuterio Gonzalez, Monterrey, Mexico, ⁵Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, Mexico, ⁶Hospital Universitario \"Dr. José Eleuterio González\", Monterrey, Nuevo León, Mexico, ⁷UANL Hospital Universitario, Monterrey, Nuevo León, Mexico
Background/Purpose: Idiopathic inflammatory myopathies (IIM), also known as myositis, are a group of heterogeneous autoimmune disorders that lead to muscle injury. Autoantibodies help make the…
Abstract Number: 1166 • ACR Convergence 2024
Characteristics, Treatments, and Outcomes of Patients with Dermatomyositis from Two Large, Nationwide US Cohorts
Michael George¹, Thomas Riley², Ellen Romich³, Bryant England⁴, Jessica Chung⁵, Karim Masri⁶, Shanette Daigle⁷, Emily Holladay⁸, Yujie Su⁹, Fenglong xie¹⁰ and Jeffrey Curtis¹¹, ¹University of Pennsylvania, Philadelphia, PA, ²Hopsital of the University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania, Media, PA, ⁴University of Nebraska Medical Center, Omaha, NE, ⁵Pfizer, New York, NY, ⁶Pfizer Inc, Collegeville, PA, ⁷FASTER, Birmingham, AL, ⁸University of Alabama at Birmingham, Edmond, OK, ⁹Illumination Health, Hoover, AL, ¹⁰University of Alabama at Birmingham; Illumination Health, Birmingham, AL, ¹¹Illumination Health, Birmingham, AL
Background/Purpose: Studies of dermatomyositis (DM) are frequently limited to single center cohorts at academic centers. We aimed to use two large nationally representative US cohorts…
Abstract Number: 1750 • ACR Convergence 2024
Safety and Preliminary Efficacy of CD19 CAR-T Cell Treatment in Rheumatic Disease – Data from the First Part of the Phase I/II CASTLE Basket Study
Georg Schett¹, Fabian Müller², Melanie Hagen³, Andreas Wirsching³, Daniela Bohr⁴, Christina Bergmann⁵, Carlo Tur³, Simon Völkl⁶, Michael Aigner⁷, Sascha Kretschmann⁷, Silvia Spoerl⁷, Soraya Kharboutli⁷, Ingrid Vasova⁷, Daniel Aletaha⁸, Hans Kiener⁹, Gerlando Natalello¹⁰, Franco Locatelli¹¹, Maria Antonietta D´Agostino¹², Aline Bozec³, Ricardo Grieshaber-Bouyer¹³ and Andreas MAckensen¹⁴, ¹Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ²Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ³Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ⁴Department of Internal Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Deutsches Zentrum für Immuntherapie (DZI), Friedrich Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ⁵Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁶Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ⁷Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁸Department of Medicine III, Division of Rheumatology, Medical University of Vienna, Wien, Austria, ⁹Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria, Vienna, Austria, ¹⁰Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ¹¹IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ¹²Catholic University of Sacred Heart, Rome, Italy, Rome, Italy, ¹³Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ¹⁴Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Bayern, Germany
Background/Purpose: Systemic autoimmune diseases are based on an aberrant activation of B cells. Autologous CD19 chimeric antigen receptor (CAR) T cells allow deep depletion of…
Abstract Number: 2079 • ACR Convergence 2024
Understanding Relapse Patterns in Anti-HMGCR Statin-induced IMNM: A Detailed Comparative Study
Silvia Grazzini¹, Angeles Galindo-Feria², Edoardo Conticini¹, Ingrid Lundberg³ and Maryam Dastmalchi⁴, ¹Università di Siena, Siena, Italy, ²Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, ³Karolinska Universitetssjukhuset, Karolinska Institutet, Stockholm, Sweden, ⁴Karolinska University hospital, Stockholm, Sweden
Background/Purpose: Statin-induced immune-mediated necrotizing myopathy (IMNM) is a recently recognized subtype of idiopathic inflammatory myopathy (IIM). This condition is marked by the sudden onset of…

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