Abstracts tagged "Myositis"

Abstract Number: 2578 • ACR Convergence 2023
Machine Learning Identifies New Sporadic Inclusion Body Myositis Endotypes Associated with Unique Autoantibody Profiles
Jenny Wei¹, Mark Tarnopolsky², Marie Hudson³, Ross Mitchell⁴, Katherine Buhler⁵, Antoine Dufour⁶, Luiz de Almeida⁶, Paul R. Fortin⁷, Eric Boilard⁸, Yann Becker⁸, Erin Hatcher², Mei Feng Zhang⁶, Marvin Fritzler⁶ and May Choi⁶, ¹University of Toronto, Toronto, ON, Canada, ²McMaster University, Hamilton, ON, Canada, ³McGill University, Montréal, QC, Canada, ⁴University of Alberta, Edmonton, AB, Canada, ⁵University of Calgary; Cumming School of Medicine, Calgary, AB, Canada, ⁶University of Calgary, Calgary, AB, Canada, ⁷Centre ARThrite - CHU de Québec - Université Laval, Quebec City, QC, Canada, ⁸Université Laval, Quebec City, QC, Canada
Background/Purpose: Sporadic inclusion body myositis (sIBM) is a subset of autoimmune inflammatory myopathies (AIM) that is often challenging to diagnose. The objective of this study…
Abstract Number: 0287 • ACR Convergence 2023
6-Minute Walk Distance Associates with Physiologic Measures and Physician/Patient Reported Outcomes in Myositis Related Interstitial Lung Disease
Sangmee Bae¹, Fereidoun Abtin², Grace Kim³, Daniela Markovic⁴, Siamak Moghadam-Kia⁵, Chester V. Oddis⁶, Lila Pourzand², Didem Saygin⁶, Daniel Sullivan⁷, Koichi Yamaguchi⁸, Donald Tashkin³, Christina Charles-Schoeman⁹, Jonathan Goldin³ and Rohit Aggarwal⁶, ¹UCLA Rheumatology, Los Angeles, CA, ²UCLA Radiological Sciences, Los Angeles, CA, ³University of California Los Angeles, Los Angeles, CA, ⁴UCLA Department of Medicine Statistics Core, Los Angeles, CA, ⁵University of Pittsburgh Medical Center, Pittsburgh, PA, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷UCLA Pulmonology, Pittsburgh, PA, ⁸University of Pittsburgh Medical Center Rheumatology, Pittsburgh, PA, ⁹UCLA Medical Center, Santa Monica, CA
Background/Purpose: The 6 minute walk distance (6MWD) provides a global evaluation of sub-maximal exercise capacity that is easily performed and highly reproducible, and is used…
Abstract Number: 0664 • ACR Convergence 2023
Quantitative Hand Held Dynamometry to Assess Muscle Strength in Scleroderma Associated Myopathy
Julie Paik¹, Christopher Mecoli², Anshula Nallapati¹, Ami Shah³, Fred Wigley⁴, Zsuzsanna McMahan⁵ and Laura Hummers¹, ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, ⁴Department of Rheumatology, Johns Hopkins Medicine, Baltimore, MD, ⁵Johns Hopkins Rheumatology, Lutherville, MD
Background/Purpose: Manual muscle strength testing can be challenging in SSc associated myopathy (SSc-AM) due to contractures or skin tightening. Since there is currently no validated…
Abstract Number: 1169 • ACR Convergence 2023
All-cause Mortality and Risk Factors for Death in a Large Multi-center Prospective Registry Cohort of Idiopathic Inflammatory Myositis in China
Liying Peng¹, Peng Yin², Mucong Li³, Qian Wang⁴, Yanhong wang¹, Shuang Zhou⁴, chanyuan wu⁴, Jiuliang zhao¹, Dong xu⁵, Mengtao Li³ and xiaofeng Zeng⁴, ¹Peking Union Medical College, Beijing, China, ²Chinese Center for Disease Control and Prevention, Beijing, China, ³Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China, ⁴Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China, ⁵Peking Union Medical College Hospital, Beijing, China
Background/Purpose: Our study depicted the mortality and independent risk factors of IIM patients in a large multi-center prospective registry cohort in China. Methods: Patients registered…
Abstract Number: 1927 • ACR Convergence 2023
Manifestations, Management and Outcomes of Interstitial Lung Disease Associated with Anti-synthetase Syndrome: A Systematic Literature Review
Koushan Kouranloo¹, Mrinalini Dey², Veronica Yioe³, Lisa G Spencer³ and Caroline V Cotton⁴, ¹Rheumatology & Internal Medicine Trainee- Surrey, UK. Education fellow University of Liverpool, Liverpool, United Kingdom, ²Queen Elizabeth Hospital, London, United Kingdom; University of Liverpool, Liverpool, United Kingdom, ³Liverpool Regional Lung Disease Service, Aintree Hospital, Lower Lane, Liverpool, United Kingdom, ⁴Department of rheumatology, Liverpool University NHS Foundation Trust, Liverpool, United Kingdom
Background/Purpose: Anti-synthetase syndrome (ASS) is a chronic autoimmune inflammatory condition, characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon and mechanic's hands. This systematic…
Abstract Number: 1965 • ACR Convergence 2023
Discordance Between Patient and Physician Perception of Disease Activity Among Patients with Idiopathic Inflammatory Myopathies: Results from the COVAD Study
Mrinalini Dey¹, Rudra Prosad Goswami², Parikshit Sen³, Samuel Shinjo⁴, Nelly Ziade⁵, Ioannis Parodis⁶, Mrudula Joshi⁷, Marcin Milchert⁸, Abraham Edgar Gracia-Ramos⁹, Lorenzo Cavagna¹⁰, Vishwesh Agarwal¹¹, Johannes Knitza¹², Jessica Day¹³, Hector Chinoy¹⁴, COVAD Study Group¹⁵, Vikas Agarwal¹⁶, Rohit Aggarwal¹⁷ and Latika Gupta¹⁸, ¹Queen Elizabeth Hospital, London, United Kingdom; University of Liverpool, Liverpool, United Kingdom, ²Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India, ³Maulana Azad Medical College, 2-Bahadurshah Zafar Marg, New Delhi, Delhi-110002, India., Dalhi, India, ⁴Faculdade de Medicina FMUSP, Universidade de Sao Paulo, São Paulo, Brazil, ⁵Saint-Joseph University, Beirut, Lebanon, ⁶Karolinska Institutet, Stockholm, Sweden, ⁷Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, India, ⁸Department of Internal Medicine, Rheumatology, Diabetology, Geriatrics and Clinical Immunology, Pomeranian Medical University in Szczecin, Szczecin, Poland, ⁹Department 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, ¹⁰Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, ¹¹Mahatma Gandhi Missions Medical College, Lucknow, India, ¹²Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, ¹³Walter and Eliza Hall Institute, Melbourne, Australia, ¹⁴The University of Manchester, Sale, United Kingdom, ¹⁵-, -, ¹⁶Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, India, ¹⁷University of Pittsburgh, Pittsburgh, PA, ¹⁸Royal Wolverhampton Trust, Wolverhampton/University of Manchester, United Kingdom
Background/Purpose: Disease activity assessment is key in the management of patients with idiopathic inflammatory myopathies (IIM). However, patients' perception of disease may differ from clinicians.…
Abstract Number: 2579 • ACR Convergence 2023
Validation of PROMIS in Adult and Pediatric Patients with Idiopathic Inflammatory Myopathies
Emma Austenfeld¹, Sara Sabbagh¹, Melodee Liegl¹, Ke Yan¹, Julie Fuller², Kelly Rouster-Stevens³, Lisa Rider⁴ and Adam Schiffenbauer⁴, ¹Medical College of Wisconsin, Wauwatosa, WI, ²UT Southwestern and Scottish Rite for Children, Dallas, TX, ³Emory University/Children's Healthcare of Atlanta, Norcross, GA, ⁴NIEHS, NIH, Bethesda, MD
Background/Purpose: This study aimed to validate the Patient Reported Outcome Measurement Information System (PROMIS) tools in patients with adult and juvenile idiopathic inflammatory myopathies (IIM/JIIM)…
Abstract Number: 028 • 2023 Pediatric Rheumatology Symposium
Achieving Medication-Free Remission in Juvenile Dermatomyositis
Harneet Ghumman¹, Ilaria Maccora², Hermine Brunner¹, Amy Cassedy³, Mekibib Altaye², Asra Firdous¹, Alexei Grom¹, Daniel Lovell¹, Angela Merritt¹, Megan Quinlan-Waters¹ and Sheila Angeles-Han², ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Cincinnati Children's Hospital, Cincinnati, OH, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Juvenile dermatomyositis (JDM) is characterized by symmetric proximal muscle weakness, distinct rash, and a risk for calcinosis. Systemic immunosuppression is needed. Evidence is limited…
Abstract Number: 064 • 2023 Pediatric Rheumatology Symposium
Towards the Development of Composite Parent-Centered Disease Activity Scores for Juvenile Dermatomyositis
Silvia Rosina¹, Ana Isabel Rebollo-Giménez², Letizia Tarantola³, Roberta Naddei⁴, Alessandro Consolaro², Angela Pistorio⁵ and Angelo Ravelli⁶, ¹IRCCS Istituto Giannina Gaslini, Genova, Italy, ²IRCCS Istituto Giannina Gaslini, UOC Reumatologia e Malattie Infiammatorie, Genova, Italy, ³Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genova, Italy, ⁴Università degli Studi di Napoli Federico II, Dipartimento di Scienze Mediche Traslazionali, Napoli, Italy, ⁵IRCCS Istituto Giannina Gaslini, Direzione Scientifica, Genova, Italy, ⁶IRRCS Istituto Giannina Gaslini and Università degli Studi di Genova, Genova, Italy
Background/Purpose: Increasing attention has been recently paid to the development of parent- and child-centered composite DAS for the assessment of health status of children with…
Abstract Number: 100 • 2023 Pediatric Rheumatology Symposium
The Brazilian Registry of Juvenile Dermatomyositis (JDM): II – A Longitudinal Assessment of Muscle Strength by Manual Muscle Test (MMT) and Childhood Myositis Assessment Scale (CMAS) Tools
Darcisio Antonio¹, Taciana Fernandes¹, Adriana Elias², Teresa Robazzi³, Ana Julia Moraes⁴, Sheila Oliveira⁵, Flavio Sztajnbok⁶, Luciana Carvalho⁷, Luciana Marques⁸, Silvana Sacchetti⁹, Maria Teresa Terreri¹⁰, Simone Appenzelle¹¹, Roberto Marini¹², Carlos Rabello Jr¹³, Cristina Magalhaes¹⁴, Melissa Fraga¹⁵, Marcia Bandeira¹⁶, Iloite Scheibel¹⁷, Isabela Daud², Beatriz Carneiro², Claudio Len¹⁸, Clovis Silva¹⁹ and Claudia Magalhaes²⁰, ¹Universidade Estadual Paulista (UNESP) Botucatu, Brazil, ²Instituto da Criança - Universidade de São Paulo (USP), São Paulo, Brazil, ³Universidade Federal da Bahia, Brazil, ⁴Universidade Federal do Para, Brazil, ⁵Universidade Federal do Rio de janeiro, Rio de Janeiro, ⁶Universidade Estadual do Rio de Janeiro, Rio de Janeiro, Brazil, ⁷Universidade de Sao Paulo- Ribeirao Preto, São Paulo, Brazil, ⁸Hospital Albert Sabin, Brazil, ⁹Santa Casa de Sao Paulo, Brazil, ¹⁰UNIFESP, São Paulo, Brazil, ¹¹University of Campinas, Campinas, Sao Paulo, Brazil, ¹²UNICAMP, São Paulo, Brazil, ¹³Hospital Geral de Fortaleza, Brazil, ¹⁴Hospital Jose de Alencar - Brasilia, Brazil, ¹⁵Hospital Darcy Vargas, Brazil, ¹⁶Hospital Pequeno Principe- Curitiba, Brazil, ¹⁷Hospital Conceição de Porto Alegre, Brazil, ¹⁸Universidade Federal de São Paulo - Unifesp, São Paulo, Brazil, ¹⁹Universidade de São Paulo, São Paulo, Brazil, ²⁰São Paulo State University, Pediatric Rheumatology Division, Botucatu, Brazil
Background/Purpose: Muscle weakness is often progressive and persistent in Juvenile Dermatomyositis (JDM). Muscle strength testing is useful for evaluating severity of muscle weakness. There is…
Abstract Number: 120 • 2023 Pediatric Rheumatology Symposium
The Brazilian Registry of Juvenile Dermatomyositis (JDM): I- Onset Clinical Features and Disease Activity Scores by DAS-20 over 2-Years-Follow Up
Beatriz Carneiro¹, Adriana Elias¹, Teresa Robazzi², Ana Julia Moraes³, Sheila Oliveira⁴, Flavio Sztajnbok⁵, Luciana Carvalho⁶, Luciana Marques⁷, Silvana Sacchetti⁸, Maria Teresa Terreri⁹, Simone Appenzelle¹⁰, Roberto Marini¹¹, Andre Cavalcante¹², Marcia Bandeira¹³, Cristina Magalhaes¹⁴, Melissa Fraga¹⁵, Iloite Scheibel¹⁶, Isabela Daud¹, Darcisio Antonio¹⁷, Claudio Len¹⁸, Clovis Silva¹⁹, Taciana Fernandes¹⁷ and Claudia Magalhaes²⁰, ¹Instituto da Criança - Universidade de São Paulo (USP), São Paulo, Brazil, ²Universidade Federal da Bahia, Brazil, ³Universidade Federal do Para, Brazil, ⁴Universidade Federal do Rio de janeiro, Rio de Janeiro, ⁵Universidade Estadual do Rio de Janeiro, Rio de Janeiro, Brazil, ⁶Universidade de Sao Paulo- Ribeirao Preto, São Paulo, Brazil, ⁷Hospital Albert Sabin, Brazil, ⁸Santa Casa de Sao Paulo, Brazil, ⁹UNIFESP, São Paulo, Brazil, ¹⁰University of Campinas, Campinas, Sao Paulo, Brazil, ¹¹UNICAMP, São Paulo, Brazil, ¹²Hospital Materno-Infantil de Goiania, Brazil, ¹³Hospital Pequeno Principe- Curitiba, Brazil, ¹⁴Hospital Jose de Alencar - Brasilia, Brazil, ¹⁵Hospital Darcy Vargas, Brazil, ¹⁶Hospital Conceição de Porto Alegre, Brazil, ¹⁷Universidade Estadual Paulista (UNESP) Botucatu, Brazil, ¹⁸Universidade Federal de São Paulo - Unifesp, São Paulo, Brazil, ¹⁹Universidade de São Paulo, São Paulo, Brazil, ²⁰São Paulo State University, Pediatric Rheumatology Division, Botucatu, Brazil
Background/Purpose: A national registry was set up, enrolling new onset JDM cases in 18 hospitals, during 3-years (2015-2018) with 2-years follow up, in a low…
Abstract Number: L10 • ACR Convergence 2022
Efgartigimod Prevents Necrosis and Allows for Muscle Fiber Regeneration in a Humanized Mouse Model of Immune-mediated Necrotizing Myopathy (IMNM)
Sarah Julien¹, Emma Briand¹, Bas van der Woning², Leentje de ceuninck³, Rachid zoubari¹, olivier benveniste⁴, laurent drouot¹ and olivier boyer⁵, ¹INSERM U1234, PAn’THER FOCIS Center of Excellence, Rouen, France, ²argenx, Boston, MA, ³argenx, Ghent, Belgium, ⁴AP-HP, Pitié-Salpêtrière University Hospital, Department of Internal Medicine and Clinical Immunology, Paris, France, ⁵INSERM U1234, PAn’THER FOCIS Center of Excellence; Rouen University Hospital, Department of Immunology and Biotherapy, Rouen, France
Background/Purpose: Immune-mediated necrotizing myopathy (IMNM) is a severe form of myositis characterized by muscle weakness and elevated creatine kinase levels in serum. The most frequent…
Abstract Number: 0155 • ACR Convergence 2022
Validity of the Mawdsley Calcinosis Questionnaire in Adult and Juvenile Dermatomyositis (DM, JDM) Patients with Calcinosis
Sarvar Nazir¹, Kelly Rouster-Stevens², Julie Fuller³, Hanna Kim⁴, Vy Do⁵, Rita Volochayev⁶, Anna Jansen⁶, Nastaran Bayat⁷, Lisa G Rider⁸ and Adam Schiffenbauer⁶, ¹National Institutes of Health, Charlotte, NC, ²Emory University/Children's Healthcare of Atlanta, Atlanta, GA, ³UT Southwestern, Frisco, TX, ⁴Division 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, ⁵UT Austin Dell Medical School, Austin, TX, ⁶National Institutes of Health, Bethesda, MD, ⁷Social & Scientific Systems, Inc., a DLH Holdings Corp (DLH) company, Bethesda, MD, ⁸Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD
Background/Purpose: Calcinosis is a complication of the idiopathic inflammatory myopathies (IIM) in which calcium salts are deposited in and around soft tissue, which can impact…
Abstract Number: 0178 • ACR Convergence 2022
Muscle Fibres Play a Critical Role in Therapeutic Response of Myositis to Glucocorticoids Through Polarisation of the Inflammatory Infiltrate by a Paracrine Mechanism
Margherita Giannini¹, Daniela Rovito², Léa Debrut³, Céline Keime⁴, Beatrice Lannes⁵, Anne-Laure Charles³, Delphine Duteil², Bernard Geny⁶, Daniel Metzger², Gilles Laverny² and Alain Meyer¹, ¹Service de Physiologie et explorations fonctionnelles, CHU, Strasbourg ; Centre de Référence des Maladies Autoimmunes Rares, CHU, Strasbourg ; UR3072, Centre de Recherche en Biomédecine, University of Strasbourg, Strasbourg, France, ²University of Strasbourg, CNRS UMR7104, INSERM U1258, IGBMC, F-67400, Illkirch, France, ³UR3072 Centre de Recherche en Biomédecine, University of Strasbourg, Strasbourg, France, ⁴University of Strasbourg, CNRS UMR 7104, INSERM UMR-S 1258, IGBMC, F-67400, Illkirch, France, ⁵Département de pathologie, Centre de référence des maladies neuromusculaires, CHU Strasbourg, University of Strasbourg, Strasbourg, France, ⁶Service de Physiologie et explorations fonctionnelles, CHU, Strasbourg ; UR3072 Centre de Recherche en Biomédecine, University of Strasbourg, Strasbourg, France
Background/Purpose: Glucocorticoids (GC) are the first line treatment for myositis patients. They improve muscle strength, yet muscle recovery is generally partial and long lasting treatment…
Abstract Number: 1864 • ACR Convergence 2022
Hepatitis B Exposure and Infection in Idiopathic Inflammatory Myopathies: Prevalence, Presentation, and Treatment Response
Ana Valle¹, Xianhong Xie² and Shereen Mahmood³, ¹Montefiore Medical Center, New York, NY, ²Department of Epidemiology & Population Health/Albert Einstein College of Medicine, Bronx, NY, ³Albert Einstein College of Medicine/Montefiore Medical Center, New York, NY
Background/Purpose: Hepatitis B (HBV) screening in patients with autoimmune conditions is vital prior to the initiation of immunosuppressive therapy given the risk of HBV reactivation.…

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