Abstracts tagged "Myopathies"

Abstract Number: 2237 • ACR Convergence 2022
Randomized Placebo-Controlled Trial to Evaluate Efficacy and Safety of Subcutaneous Abatacept in Adults with Active Idiopathic Inflammatory Myopathy: Results of the 24-Week Double-Blind and 28-Week Open-Label Periods
Rohit Aggarwal¹, Ingrid Lundberg², Yeong-Wook Song³, Aziz Shaibani⁴, Victoria Werth⁵ and Michael Maldonado⁶, ¹Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ²Karolinska Universitetssjukhuset, Karolinska Institutet, Stockholm, Sweden, ³Seoul National University, Seoul, Republic of Korea, ⁴Nerve and Muscle Center of Texas, Houston, TX, ⁵Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, ⁶Bristol Myers Squibb, Princeton, NJ
Background/Purpose: A 52-week (wk), randomized, placebo (PBO)-controlled phase 3 trial of subcutaneous (SC) abatacept (ABA) and standard of care (SOC) was performed in patients (pts)…
Abstract Number: 0175 • ACR Convergence 2022
Extracorporeal Membrane Oxygenation for Acute Lung Injury in Idiopathic Inflammatory Myopathies – a Potential Lifesaving Intervention
boyang zheng¹, Ellen Eline², Lilian Xu³, Kun Huang⁴, Greet Hermans⁵, michael Perch⁶, gordan Samoukovic¹, ellen De Langhe⁵, Maryam Dastmalchi⁷, Lisa Christopher-Stine², Louise Diederichsen⁸ and Valerie Leclair¹, ¹McGill University, Montréal, QC, Canada, ²Johns Hopkins University, Baltimore, MD, ³Johns Hopkins University School of Medicine, Baltimore, MD, ⁴University of British Columbia, Vancouver, BC, Canada, ⁵University Hospitals Leuven, Leuven, Belgium, ⁶Righospitalet, University of Copenhagen, Copenhagen, Denmark, ⁷Karolinska University Hospital, Stockholm, Sweden, ⁸Copenhagen University Hospital, Rigshospitalet, Dragør, Denmark
Background/Purpose: Idiopathic inflammatory myopathies (IIM) can present with acute IIM-related lung injury and respiratory failure, leading to a high mortality risk in intensive care units…
Abstract Number: 2242 • ACR Convergence 2022
Metabolomics-based Identification of Metabolic Dysfunction in Inclusion Body Myositis
Jemima Albayda¹, Cissy Zhang¹, Pratik Khare¹, Brittany Adler¹, Erika Darrah¹, Lisa Christopher-Stine¹, Thomas E Lloyd² and Anne Le¹, ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: Sporadic Inclusion Body Myositis (IBM) is the most common myopathy over the age of 50 and is currently refractory to treatment. The pathogenesis of…
Abstract Number: 0176 • ACR Convergence 2022
Diagnostic Accuracy and Clinical Relevance of Muscle Biopsy in Patients with Suspected Myositis
Kastriot Kastrati¹, Nasim NAKHOST LOTFI², Marwa Tawfik³, Ellen Gelpi², Romana Hoeftberger², Hans-Peter Kiener², Daniel Aletaha⁴, Michael Bonelli⁵ and Helga Radner⁵, ¹Division of Rheumatology, Vienna, Austria, ²Medical University Vienna, Vienna, Austria, ³Suez Canal University, Ismailia, Egypt, ⁴Medical University Vienna, Wien, Austria, ⁵Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
Background/Purpose: Diagnosis of idiopathic inflammatory myopathies (IIM) represents a clinical challenge and muscle biopsy is regarded as a fundamental procedure for confirming diagnosis. However, the…
Abstract Number: 0441 • ACR Convergence 2021
A Randomized, Double-blind, Placebo-controlled Study of Arimoclomol in Patients with Inclusion Body Myositis
Pedro Machado¹, Richard Barohn², Michael McDermott³, Thomas Blaetter⁴, Tom Lloyd⁵, Aziz Shaibani⁶, Miriam Freimer⁷, Anthony Amato⁸, Emma Ciafaloni³, Sarah Jones⁹, Tahseen Mozaffar¹⁰, Summer Gibson¹¹, Matthew Wicklund¹², Todd Levine¹³, Claus Sundgreen⁴, Tim Carstensen⁴, Karen Bonefeld⁴, Anders Jørgensen⁴, Karina Phonekeo⁴, Andrew Heim¹⁴, Laura Herbelin¹⁴, Michael Hanna¹⁵ and Mazen Dimachkie¹⁴, ¹Centre for Rheumatology & Department of Neuromuscular Diseases, University College London, London, United Kingdom, ²University of Missouri - Columbia, Columbia, MO, ³University of Rochester, Rochester, NY, ⁴Orphazyme A/S, Copenhagen, Denmark, ⁵Johns Hopkins University, Baltimore, MD, ⁶Nerve and Musle Center, Houston, TX, ⁷Ohio State University, Columbus, OH, ⁸Brigham and Women's Hospital, Boston, MA, ⁹University of Virginia, Charlottesville, VA, ¹⁰University of California Irvine, Irvine, CA, ¹¹University of Utah, Salt Lake City, UT, ¹²University of Colorado, Denver, CO, ¹³Phoenix Neurological Associates, Phoenix, AZ, ¹⁴University of Kansas Medical Center, Kansas City, KS, ¹⁵University College London, London, United Kingdom
Background/Purpose: Inclusion body myositis (IBM) is the most common idiopathic inflammatory myopathy occurring in patients over the age of 45 years. Since immune suppression has…
Abstract Number: 0443 • ACR Convergence 2021
Safety and Efficacy of Belimumab in the Treatment of Adult Idiopathic Inflammatory Myositis (Polymyositis and Dermatomyositis)
Priyal Chadha¹, Sonali Narain², Preeya Nandkumar³, Cristine Sison⁴, Elena Schiopu⁵, Todd Levine⁶, James Tsang⁷ and Galina Marder⁸, ¹SUNY Upstate Medical University, Syracuse, NY, ²Northwell Health, Great Neck, NY, ³Division of Rheumatology, Department of Medicine, Northwell, Great Neck, NY, ⁴Biostatistics Unit , Department of Molecular Medicine, Feinstein Institutes for Medical Research at Northwell Health, Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, ⁵University of Michigan, Ann Arbor, MI, ⁶Phoenix Neurological Associates, Phoenix, AZ, ⁷Center for Research Informatics & Innovation (CRII), Feinstein Institute for Medical Research at Northwell Health, Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, ⁸Division of Rheumatology, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY
Background/Purpose: Treatment of Idiopathic Inflammatory Myositis (IIM) includes steroids and immunosuppressive agents. Targeting IL6, IL1, TNF for treatment of IIM has not been successful, and…
Abstract Number: 0697 • ACR Convergence 2021
Detection of Autoantibodies Against Muscle-Specific Four-and-a-Half-LIM Domain 1 (FHL1) in Inflammatory Myopathies: Results from a Single-Center Cohort
Angeles Shunashy Galindo-Feria¹, Begum Horuluoglu¹, Jessica Day², Catia Cerqueira³, Edvard Wigren⁴, Susanne Gräslund⁴, Susanna Proudman⁵, Ingrid E Lundberg⁴ and Vidya Limaye⁶, ¹Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, ²Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia and Discipline of Medicine, University of Adelaide, Adelaide, Australia, ³4Dcell, Montreuil, France, ⁴Karolinska Institutet, Karolinska University Hospital, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden, Stockholm, Sweden, ⁵University of Adelaide, Medindie, Australia, ⁶Royal Adelaide Hospital, Adelaide, Australia
Background/Purpose: Autoantibodies targeting a muscle-specific autoantigen, four-and-a-half-LIM-domain 1 (FHL1), have been previously identified in patients with idiopathic inflammatory myopathies (IIM) (1). The aim of this…
Abstract Number: 0701 • ACR Convergence 2021
Use and Yield of Computed Tomography as a Cancer Surveillance Method in Idiopathic Inflammatory Myositis
Christopher Mecoli¹, Brant Chee², XingYao Wang³, Mengkun Chen⁴, William Kelly³, Elizabeth Platz⁴, Livia Casciola-Rosen⁵, Lisa Christopher-Stine³ and Ami Shah⁶, ¹Johns Hopkins University School of Medicine, Baltimore, MD, ²Applied Physics Laboratory, Baltimore, MD, ³Johns Hopkins University, Baltimore, MD, ⁴Johns Hopkins School of Public Health, Baltimore, MD, ⁵Johns Hopkins, Baltimore, MD, ⁶Johns Hopkins Rheumatology, Baltimore, MD
Background/Purpose: To inform guidance for cancer surveillance in patients with idiopathic inflammatory myositis (IIM), we conducted a retrospective cohort study in a single tertiary referral…
Abstract Number: 0704 • ACR Convergence 2021
Anti-SSa/SSb and Ro52 and Interstitial Lung Disease in Idiopathic Inflammatory Myopathies
Sonali Narain¹, Ana Valle², Maria-Louise Barilla-Labarca¹ and Galina Marder³, ¹Northwell Health, Great Neck, NY, ²Montefiore Medical Center/Albert Einstein College of Medicine, New York, NY, ³Division of Rheumatology, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY
Background/Purpose: In idiopathic inflammatory myopathies (IIM), SSa/SSb and Ro52 antibodies are frequently reported, especially in association with interstitial lung disease (ILD). However, limited literature exists…
Abstract Number: 0706 • ACR Convergence 2021
Mycophenolate Mofetil and Methotrexate Efficacy in Dermatomyositis
Madison Grinnell¹, Emily Keyes², DeAnna Diaz², Thomas Vazquez³, Rui Feng⁴ and Victoria Werth², ¹Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania and Philadelphia VAMC, OMAHA, NE, ²Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania and Philadelphia VAMC, Philadelphia, PA, ³FIU Wertheim College of Medicine, Virginia Beach, VA, ⁴University of Pennsylvania Department of Biostatistics, Philadelphia, PA
Background/Purpose: Treatment of dermatomyositis (DM) typically follows a stepwise sequence starting with either methotrexate (MTX) or mycophenolate mofetil (MMF) after an inadequate response to antimalarial…
Abstract Number: 0707 • ACR Convergence 2021
Association of Pneumomediastinum with Poor Prognosis in Patients with Myositis-Associated Interstitial Lung Disease
Kazuki Hirano¹, Azusa Kojima¹, Akira Ishii¹, Mai Sugiyama¹, Yuto Izumi¹, Noriko Sasaki², Yuji Hosono¹, Chiho Yamada¹ and Shinji Sato³, ¹Tokai University School of Medicine, Isehara, Japan, ²tokai university, sagamihara-city, Japan, ³Tokai University, Isehara, Japan
Background/Purpose: Pneumomediastinum is an important complication in patients with myositis-associated interstitial lung disease (ILD). Patients with myositis and ILD who had pneumomediastinum during the disease…
Abstract Number: 0712 • ACR Convergence 2021
Immune Checkpoint Inhibitor-related Myotoxicity : Musculoskeletal and/or Neuromuscular Junction Disorder ?
Hortense Chassepot¹, Lotta Plomp², Dimitri Psimaras³, Thierry Maisonobe³, Sarah Leonard Louis⁴, Isabelle Plu⁴, Mehdi Touat³, Celine Anquetil⁵, Nadege Wesner⁵, Nicolas Champtiaux⁵, Aude Rigolet⁵, Sophie Demeret³, Nicolas Weiss³, Baptiste Abbar⁶, Marie-Claire Bretagne⁷, Bruno Pinna⁷, Capucine Morelot⁸, Martin Dres⁸, Thomas Similowski⁸, Stephane Ederhy⁹, Joe-Elie Salem⁷, Olivier Benveniste⁵ and Yves Allenbach⁵, ¹Universite de Picardie Jules Verne UPJV - Amiens Picardie Hospital, Department of Clinical Immunology and Internal Medicine, Fourqueux, France, ²Amsterdam University Medical Center (AUMC), Amsterdam, Netherlands, ³Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Neurology, Paris, France, ⁴Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Neuropathology, Paris, France, ⁵Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Clinical Immunology and Internal Medicine, Paris, France, ⁶Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Oncology, Paris, France, ⁷Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Pharmacology, Paris, France, ⁸Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Pneumology - R3S, Paris, France, ⁹Sorbonne University - AP-HP, Pitié-Salpêtrière Hospital, Department of Cardiology, Paris, France
Background/Purpose: Immune checkpoint inhibitor (ICI)-related adverse events (irAE) recently emerged as new diseases in the field of auto-immunity. Among them, ICI-related myotoxicity has the highest…
Abstract Number: 0718 • ACR Convergence 2021
Pain Severity and Interference in Adult Autoimmune Inflammatory Myopathies
Harmony Tsui¹, Mianbo Wang², Mathieu Piché³, Alexandra Ladouceur⁴, Evelyne Vinet⁵, Alexandra Albert⁶, Maggie Larche⁷, Annaliese Tisseverasinghe⁸, Marie Hudson¹ and Valerie Leclair⁹, ¹McGill University, Montréal, QC, Canada, ²Lady Davis institute for Medical Research, Montréal, QC, Canada, ³Université du Québec à Trois-Rivières, Trois-Rivières, QC, Canada, ⁴McGill University Health Center, Montréal, QC, Canada, ⁵McGill University Health Centre, Mont Royal, QC, Canada, ⁶CHU de Quebec, Québec City, QC, Canada, ⁷McMaster University, Hamilton, ON, Canada, ⁸University of Manitoba, Winnipeg, MB, Canada, ⁹McGill University, Karolinska Insitutet, Montréal, QC, Canada
Background/Purpose: Although pain is one of the most common and highest priority symptoms reported by people with autoimmune inflammatory myopathies (AIM), large descriptive studies on…
Abstract Number: 0995 • ACR Convergence 2020
Jo-1-Binding B Cells Undergo Limited Class-Switching but Are Biased Towards Autoreactive-Prone and Memory B Cell Subsets in Anti-histidyl-tRNA Synthetase Syndrome
Jennifer Young-Glazer¹, Alberto Cisneros², Erin Wilfong¹, Scott Smith¹, Leslie J. Crofford¹ and Rachel Bonami¹, ¹Vanderbilt University Medical Center, Nashville, TN, ²Vanderbit University Medical Center, Nashville, TN
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are systemic autoimmune diseases traditionally classified as dermatomyositis or polymyositis, but these disorders are increasingly defined by the presence of…
Abstract Number: 1098 • ACR Convergence 2020
Anti-cortactin Autoantibodies Are Associated with Key Clinical Features in Adult Myositis but Are Rarely Present in Juvenile Myositis
Iago Pinal-Fernandez¹, Katherine Pak², Albert Gil-Vila³, Andres Baucells⁴, Benjamin Plotz⁵, Maria Casal-Dominguez⁶, Assia Derfoul⁷, Maria Angeles Martinez⁴, Albert Selva-O'Callaghan³, Sara Sabbagh⁸, Frederick W. Miller¹, Lisa G. Rider⁹, Lisa Christopher-Stine¹⁰ and Andrew Mammen⁶, ¹National Institutes of Health, Bethesda, MD, ²NIAMS, NIH, Bethesda, MD, ³Vall d’Hebron Hospital, Barcelona, Spain, ⁴Sant Pau Hospital, Barcelona, Spain, ⁵Division of Rheumatology, New York University Langone Health, New York, NY, ⁶NIH, Bethesda, MD, ⁷NIAMS, NIH, Bethesda, ⁸Division of Rheumatology, Department of Pediatrics, Medical College of Wisconsin, Washington, DC, ⁹Environmental Autoimmunity Group, Clinical Research Branch, NIEHS, NIH, Garrett Park, MD, ¹⁰Johns Hopkins University, Baltimore, MD
Background/Purpose: To define the prevalence and clinical phenotype of anti-cortactin autoantibodies in adult and juvenile myositis.Methods: In this longitudinal cohort study, anti-cortactin autoantibody tiers were…

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