Abstracts tagged "Myopathies"

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: 0085 • ACR Convergence 2020
Classifications of Inflammatory Myopathies: Differentially Expressed Membrane-Bound Complement Regulators Allow Specific Patterns of Membrane Attack Complex Deposition
Fadi Charouf¹, Netanel Karbian¹, Itamar Altman¹, Yakov Fellig¹ and Dror Mevorach¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of heterogeneous disorders that typically present with proximal muscle weakness. According to the classification criteria, IMs are…
Abstract Number: 1086 • ACR Convergence 2020
The Diagnostic Value and Clinical Significance of Myositis-Specific Antibodies in Patients Suspected to Have Autoimmune Myopathies and/or Autoimmune Rheumatic Diseases
Sarah El Chami¹, Christopher Williams¹, Ghaith Noaiseh¹, Amrita Bath¹ and Duaa Jabari¹, ¹The University of Kansas Health System, Kansas city, KS
Background/Purpose: Myositis-specific antibodies (MSA) are thought to be highly specific in patients with idiopathic inflammatory myopathies.[1] However, in clinical practice these antibodies are frequently found…
Abstract Number: 0932 • ACR Convergence 2020
Skeletal Muscle Involvement in Systemic Sclerosis Predisposes to Severe Gastrointestinal Tract and Cardiac Muscle Disease
Reshad Mahmud¹, Lei Zhu², Maureen Laffoon², Thomas Medsger³ and Robyn Domsic⁴, ¹UPMC, Pittsburgh, PA, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh School of Medicine, Verona, PA, ⁴University of Pittsburgh School of Medicine, Pittsburgh, PA
Background/Purpose: Tissues containing muscle cells are affected in systemic sclerosis (SSc) patients in different organ systems: skeletal muscle, cardiac muscle and gastrointestinal (GI) smooth muscle. …
Abstract Number: 1088 • ACR Convergence 2020
Recruitment Rates of Virtual Remote Research (Tele-Research) in Myositis
Siamak Moghadam-Kia¹, Chester Oddis², Swamy Venturupalli³, Shalini Mahajan⁴, Daphne Scaramangas Plumley⁵, Dana Ascherman⁶, Nicole Neiman⁷, Faith Onelangsy⁷, Diane Koontz¹, Kim Goldby-Reffner⁷, Sedin Dzanco⁷, Lei Zhu⁶ and Rohit Aggarwal⁶, ¹University of Pittsburgh Medical Center, Pittsburgh, PA, ²Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, ³Cedars Sinai Medical Center, Los Angeles, ⁴Attune Health, Los Angeles, ⁵Attune Health, Los Angeles, CA, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷UPMC, Pittsburgh
Background/Purpose: There is a paucity of randomized, controlled clinical trials in myositis. Subject enrollment and retention in clinical trials for this rare, heterogeneous disease has…
Abstract Number: 0933 • ACR Convergence 2020
Advanced Multiparametric Magnetic Resonance Imaging of Muscle for Detection and Quantification of Muscle Disease in Systemic Sclerosis
Julie Paik¹, Fredrick Wigley², Laura Fayad³, Laura Hummers⁴ and Michael Jacobs¹, ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³Joh, Baltimore, MD, ⁴Johns Hopkins Univerisity, Ellicott City, MD
Background/Purpose: Skeletal myopathy in systemic sclerosis (SSc) is underappreciated yet an important manifestation of SSc. While it has been reported that there are distinct histopathologic…
Abstract Number: 1096 • ACR Convergence 2020
Assessing Interstitial Lung Disease in a Racially Diverse Population with Idiopathic Inflammatory Myositis
Jammie Law¹, Ana Valle², Katherine Mullins³ and Shereen Mahmood⁴, ¹Montefiore Medical Center, Bronx, NY, ²Albert Einstein College of Medicine, New York, NY, ³Montefiore Medical Center, BROOKLYN, NY, ⁴Albert Einstein College of Medicine / Montefiore Medical Center, Wantagh, NY
Background/Purpose: Interstitial lung disease (ILD) is a common extra-muscular manifestation of Idiopathic Inflammatory Myositis (IIM) and increases risk of mortality. Prior studies and registries have focused on either smaller cohorts or predominantly Caucasian/European populations. Our aim was to better characterize the…