Abstracts tagged "dermatomyositis"

Abstract Number: 1657 • ACR Convergence 2023
Mapping the Network of Coordinated Immune Dysregulation in Juvenile Dermatomyositis at Single-cell Resolution
Gabrielle Rabadam¹, Camilla Wibrand², Emily Flynn¹, George Hartoularos¹, Yang Sun¹, Susan Kim³, Chun Jimmie Ye¹, Zev Gartner¹, Marina Sirota⁴ and Jessica Neely¹, ¹University of California San Francisco, San Francisco, CA, ²Aarhus University, Aarhus, Denmark, and University of California San Francisco, San Francisco, CA, ³UCSF Benioff Children's Hospital, San Francisco, CA, ⁴Bakar Computational Health Sciences Institute, University of California San Francisco, San Francisco, CA
Background/Purpose: Juvenile Dermatomyositis (JDM) is a rare multisystem autoimmune condition that involves complex immune responses in both innate and adaptive compartments. Currently, we have a…
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: 0085 • ACR Convergence 2023
Fibroblasts Promote Upregulation of Cannabinoid Type 2 Receptor on Inflammatory Cells in Dermatomyositis
DeAnna Diaz¹, Muhammad Bashir¹, Rohan Dhiman², Avital Baniel¹, Julianne Kleitsch¹, Rachita Pandya¹, Meena Sharma¹, Thomas Vazquez¹, Ming-Lin Liu², Mariko Momohara² and Victoria Werth³, ¹Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania and Corporal Michael J. Crescenz VAMC, Philadelphia, PA
Background/Purpose: Dermatomyositis (DM) is a chronic, systemic autoimmune disease affecting the skin, muscle, and lungs. The activation of CB2R has been shown to reduce several,…
Abstract Number: 1163 • ACR Convergence 2023
Anti-melanoma Differentiation Associated Gene 5 (Anti-MDA5) Antibody Dermatomyositis: Clinical Features and Outcome in a Racially Diverse Patient Cohort
Sai Koyoda¹, Dawn Wahezi², Xianhong Xie³, Anand Kumthekar⁴, Clement Tagoe³ and Bibi Ayesha⁵, ¹Essen Medical Center, Rahway, NJ, ²Children's Hospital at Montefiore, New Rochelle, NY, ³Albert Einstein College of Medicine, Bronx, NY, ⁴Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY, ⁵Montefiore Medical Center, Bronx, NY
Background/Purpose: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody dermatomyositis typically expresses rapidly progressing interstitial lung disease (RP-ILD) and ulcerative skin lesions, with or without muscle involvement.…
Abstract Number: 1709 • ACR Convergence 2023
Tape Stripping Expression Signatures Identify Biologically Unique Juvenile Dermatomyositis Patient Subgroup Characterized by Increased Mitochondrial Dysfunction
Jessica Turnier, Celine Berthier, Sarah Vandenbergen, Christine Goudsmit, Madison McClune, Johann E. Gudjonsson, Lam Tsoi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Skin inflammation in juvenile dermatomyositis (JDM) can signal disease onset or flare and prevent complete disease remission. The study of cutaneous expression signatures holds…
Abstract Number: 0126 • ACR Convergence 2023
Epidemiology and Retrospective Case-Control Analysis of IIM in the US Veteran Population
Vladimir Liarski, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Idiopathic Inflammatory Myopathy (IIM) - comprising polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM) - is rare and difficult to study. We sought…
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: 1814 • ACR Convergence 2023
Silica and Other Exposures from Jobs and Hobbies Are Associated with Myositis Phenotype in a National Myositis Registry
Christine Parks¹, Jesse Wilkerson², Payam Noroozi Farhad³, Nastaran Bayat⁴, Adam Schiffenbauer⁵, Hermine Brunner⁶, Robert Goldberg⁷, Frederick Miller⁸ and Lisa Rider⁵, ¹Epidemiology Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Research Triangle Park, NC, ²NIEHS/NIH, Durham, NC, ³Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, ⁴Social Scientific Systems, DLH Holdings Corp, Silver Spring, MD, ⁵NIEHS, NIH, Bethesda, MD, ⁶Cincinnati Children's Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ⁷Myositis Foundation, Alexandria, VA, ⁸NIH, NIEHS, Chapel Hill, NC
Background/Purpose: Growing evidence suggests that environmental factors may contribute to disease phenotype among patients diagnosed with the idiopathic inflammatory myopathies (IIM), dermatomyositis (DM), polymyositis (PM),…
Abstract Number: 0285 • ACR Convergence 2023
Patient Reported Physical Function, Mental Health, and Treatment Patterns in Dermatomyositis
Lisa Christopher-Stine¹, Julie Paik¹, Brendan M. Johnson², Taryn Smith², Jolie Feldman² and Paul N. Mudd Jr.², ¹Johns Hopkins University, Baltimore, MD, ²Priovant Therapeutics, Durham, NC
Background/Purpose: Dermatomyositis (DM) is a rare, debilitating, idiopathic inflammatory myopathy characterized by painful, itchy skin rash and proximal muscle weakness that can significantly impact daily…
Abstract Number: 1165 • ACR Convergence 2023
Long-term Changes in Lung Function in Melanoma Differentiation-associated Protein 5 (MDA5) Antibody Positive Dermatomyositis Patients: Experience of a Single Center Longitudinal Cohort in North America
Jenice Cheah¹, Sangmee Bae², Tiffany De Leon², Yuna Lee¹ and Christina Charles-Schoeman³, ¹University of California Los Angeles, Los Angeles, CA, ²UCLA Rheumatology, Los Angeles, CA, ³UCLA Medical Center, Santa Monica, CA
Background/Purpose: Interstitial lung disease (ILD) associated with antibodies to melanoma differentiation-associated protein 5 (MDA5 ab) in patients with dermatomyositis (DM) has been associated with a…
Abstract Number: 1946 • ACR Convergence 2023
Zetomipzomib Demonstrates Favorable Long-term Safety and Tolerability Profile Without Signs of Immunosuppression: Results from the PRESIDIO Study and Its Open-label Extension Study in Patients with Dermatomyositis and Polymyositis
Rohit Aggarwal¹, Namita Goyal², Diana Lam³, Allen Ng³, Richard Leff⁴, Kathryn Ray³, Eunmi Park³ and Noreen Henig³, ¹University of Pittsburgh, Pittsburgh, PA, ²UCI Health, Orange, CA, ³Kezar Life Sciences, South San Francisco, CA, ⁴Kezar Life Sciences, Chadds Ford, PA
Background/Purpose: Polymyositis (PM) and dermatomyositis (DM) are chronic autoimmune diseases characterized by muscle inflammation and weakness. In a collagen-induced myositis mouse model, immunoproteasome inhibition has…
Abstract Number: 0289 • ACR Convergence 2023
Markers of Endothelial Damage Are Elevated in Patients with Dermatomyositis Associated Interstitial Lung Disease and Associated with Low Paraoxonase-1 Activity
Sangmee Bae¹, Ani Shahbazian¹, Jennifer Wang², Tiffany De Leon¹, Yuna Lee¹, srinivasa Reddy³ and Christina Charles-Schoeman⁴, ¹UCLA Rheumatology, Los Angeles, CA, ²University of California Los Angeles, Los Angeles, CA, ³UCLA Department of Molecular and Medical Pharmacology, Los Angeles, CA, ⁴UCLA Medical Center, Santa Monica, CA
Background/Purpose: Damage to the vascular endothelium is implicated in the pathogenesis of idiopathic inflammatory myopathies (IIM) and its associated interstitial lung disease (ILD), with microvascular…
Abstract Number: 1167 • ACR Convergence 2023
Transcriptome Analysis of Peripheral Blood Reveals Superiority of the Triple Combination of Baricitinib, Rituximab, and Tacrolimus Therapy (BRT-Tx.) for anti-MDA5 Antibody-positive Dermatomyositis (MDA5-DM)
Yoshinobu Koyama¹, Yoshiharu Sato², Yu Nakai³ and Moe Tokunaga(Sakamoto)³, ¹Japanese Red Cross Okayama Hospital, Okayama, Japan, ²DNA Chip Research Inc, Tokyo, Japan, ³Rheumatology, Center for Autoimmune diseases, Japanese Red Cross Okayama Hospital, Okayama, Japan
Background/Purpose: MDA5-DM is characterized by high mortality due to rapid progressive ILD. We reported that in MDA-5DM, (1) RIG-1-like receptor signaling is enhanced, (2) antiviral…
Abstract Number: 1949 • ACR Convergence 2023
Relapse of Rapidly Progressive Interstitial Lung Disease in Patients with Anti-Melanoma Differentiation-Associated Gene 5 Autoantibody-Positive Dermatomyositis
Noriko Sasaki, Toshiki Ohisa, Akira Ishii, Mai Sugiyama, Yuichiro Ota, Chiho Yamada and Shinji Sato, Tokai university School of Medicine, Isehara, Japan
Background/Purpose: Autoantibodies to Melanoma Differentiation-Associated Gene 5 (MDA5) are found specifically in patients with dermatomyositis (DM). Their presence is closely associated with rapidly progressive interstitial…
Abstract Number: 0297 • ACR Convergence 2023
Characteristics of Anti-MDA-5 Associated Dermatomyositis in Southern California with a Large Hispanic Population: Case Series
Eaman Alhassan¹, Belina Yi², Jack Rodman¹ and Leanna Wise³, ¹University of Southern California, Los Angeles, CA, ²Children's Hospital Los Angeles, Los Angeles, CA, ³LAC+USC/Keck Medicine of USC, Pasadena, CA
Background/Purpose: There is little to no data about the presentation and clinical course of anti-melanoma differentiation-associated gene-5 antibody (anti-MDA-5) dermatomyositis patients in a primarily U.S.…

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