Abstracts tagged "Myopathies"

Abstract Number: 1164 • ACR Convergence 2023
Can We Differentiate Patients with Dysferlinopathies and Inflammatory Myopathies by Muscle Ultrasound? A Discriminant Analysis Study
Carina Soto-Fajardo¹, Sinthia Solorzano Flores¹, Abish Angeles-Acuña¹, Fabian Carranza Enriquez¹, Rosa-Elena Escobar-Cedillo², Saul Renan-Leon², Karina Arias Callejas³, Alejandra Enriquez-Luna², Graciliano Ramon-Diaz² and Carlos Pineda², ¹Instituto Nacional de Rehabilitación "Luis Guillermo Ibarra Ibarra", Mexico City, Mexico, ²Instituto Nacional de Rehabilitacion, Mexico City, Mexico, ³Instituto Nacional de Rehabilitación "Luis Guillermo Ibarra Ibarra", Mexico City, Mexico
Background/Purpose: Immune-mediated myopathies (IMM) are a heterogeneous group of diseases characterized by inflammation and muscle weakness; among their differential diagnoses are the dysferlinopathies, which are…
Abstract Number: 1969 • ACR Convergence 2023
Multimorbidity and PROMIS Health Outcomes in Patients with Idiopathic Inflammatory Myopathies: Analysis from the COVAD Study
Marco Fornaro¹, Vincenzo Venerito², Florenzo Iannone³, Naveen R⁴, Elena Nikiphorou⁵, Mrudula Joshi⁶, Ai Lyn Tan⁷, Sreoshy Saha⁸, Samuel Shinjo⁹, Vishwesh Agarwal¹⁰, Nelly Ziade¹¹, Tsvetelina Velikova¹², Esha Kadam¹³, Marcin Milchert¹⁴, Ioannis Parodis¹⁵, Abraham Edgar Gracia-Ramos¹⁶, Lorenzo Cavagna¹⁷, Masataka Kuwana¹⁸, Johannes Knitza¹⁹, Ashima Makol²⁰, Dey Dzifa²¹, CARLOS ENRIQUE TORO GUTIERREZ²², CARLO VINICIO CABALLERO²³, Oliver Distler²⁴, Jessica Day²⁵, Hector Chinoy²⁶, Vikas Agarwal⁴, Rohit Aggarwal²⁷, Latika Gupta²⁸ and COVAD Study Group²⁹, ¹University of Bari, Grottaglie, Italy, ²Rheumatology Department, Università degli Studi di Bari, Bari, Italy, ³Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area, University of Bari "Aldo Moro", Bari, Italy, ⁴Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, India, ⁵King's College London, London, United Kingdom, ⁶Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, India, ⁷University of Leeds, Leeds, United Kingdom, ⁸Mymensingh Medical College, Faridpur, Bangladesh, ⁹Faculdade de Medicina FMUSP, Universidade de Sao Paulo, São Paulo, Brazil, ¹⁰Mahatma Gandhi Missions Medical College, Lucknow, India, ¹¹Saint-Joseph University, Beirut, Lebanon, ¹²Department of Clinical Immunology, Medical Faculty, University Hospital "Lozenetz", Sofia University St. Kliment Ohridski, Sofia, Bulgaria, ¹³Seth Gordhandhas Sunderdas Medical College and King Edwards Memorial Hospital, Mumbai, India, ¹⁴Department of Internal Medicine, Rheumatology, Diabetology, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Szczecin, Poland, ¹⁵Karolinska Institutet, Stockholm, Sweden, ¹⁶Department of Internal Medicine, General Hospital, National Medical Center "La Raza", Instituto Mexicano del Seguro Social, Av. Jacaranda S/N, Col. La Raza, Del. Azcapotzalco, C.P. 02990, Mexico City, Mexico, ¹⁷Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, ¹⁸Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ¹⁹Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, ²⁰Mayo Clinic, Rochester, MN, Rochester, MN, ²¹Department of Medicine and Therapeutics, University of Ghana School of Medicine and Dentistry, College of Health Sciences, Korle-Bu, Accra, Ghana, ²²Centro de Estudios de Reumatología y Dermatología SAS, Cali, Colombia, ²³REUMACARIBE IPS, Barranquilla, Colombia, ²⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²⁵Walter and Eliza Hall Institute, Melbourne, Australia, ²⁶The University of Manchester, Sale, United Kingdom, ²⁷University of Pittsburgh, Pittsburgh, PA, ²⁸Royal Wolverhampton Trust, Wolverhampton/University of Manchester, United Kingdom, ²⁹-, -
Background/Purpose: Comorbidities have a profound impact on the quality of life (QoL), though global data on the burden of comorbidities and its impact on health…
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…
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…

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