Abstracts tagged "dermatomyositis"

Abstract Number: 1255 • ACR Convergence 2023
Clinical Characteristics and Disease Outcomes of anti-NXP2 Positive Juvenile Dermatomyositis: A Single Center Cohort
Sarah Molina¹, Dawn Gist², Marietta De Guzman³, Eyal Muscal⁴, Jamie Lai² and Maria Pereira³, ¹Baylor College of Medicine - Pediatrics Residency Program, Houston, TX, ²Baylor College of Medicine / Texas Children's Hospital, Houston, TX, ³Baylor College of Medicine, Houston, TX, ⁴Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX
Background/Purpose: Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory myopathy with clinically heterogeneous presentations that can be categorized by myositis-specific antibodies (MSAs). NXP2 is among…
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: 1155 • ACR Convergence 2023
Identification of Serum Biomarkers Associated with Muscle Damage Detected on MRI in Polymyositis/dermatomyositis
Haruna Matsuo¹, Toshimasa Shimizu², Tomohiro Koga³, Nozomi Oki⁴, Mari Kamiya⁵, Natsuka Umezawa⁵, Shinsuke Yasuda⁶, Masataka Uetani⁷ and Atsushi Kawakami⁸, ¹Nagasaki University, Nagasaki City, Japan, ²Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ³Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University, Nagasaki, Japan, ⁴Department of Radiological Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki City, Japan, ⁵Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan, ⁶Department of Rheumatology, Graduate School of Medical and Dental Science, Tokyo Medical and Dental University (TMDU), Tokyo, Japan, ⁷Department of Radiological Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ⁸Nagasaki University, Nagasaki, Japan
Background/Purpose: Polymyositis/Dermatomyositis (PM/DM) is a chronic inflammatory disease characterized by muscle weakness, and cutaneous manifestations. Although PM/DM exhibits distinct disease phenotypes based on autoantibodies, assessing…
Abstract Number: 1587 • ACR Convergence 2023
Mitochondrial Z-DNA and ZBP1 Drive Autoimmune Photosensitivity
Benjamin Klein, Mack Reynolds, Bin Xu, Mehrnaz Gharaee-Kermani, Amanda Victory, Shannon Loftus, Mary O'Riordan and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Autoimmune photosensitivity is observed in type I Interferon (IFN) mediated diseases such as systemic and cutaneous lupus erythematosus (SLE/CLE) and dermatomyositis. Type I IFN…
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: 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: 004 • 2023 Pediatric Rheumatology Symposium
Analysis of Patients with Juvenile Dermatomyositis Compared to Healthy Controls Using CITE-seq Identifies Differences in Cell Composition and Gene and Epitope Expression
Camilla Wibrand¹, Emily Flynn², Gabrielle Rabadam², George Hartoularos², Yang Sun², Chun Ye², Susan Kim³, Marina Sirota² and Jessica Neely¹, ¹UCSF, San Francisco, CA, ²University of California, San Francisco, San Francisco, CA, ³UCSF Benioff Children's Hospital, San Francisco, CA
Background/Purpose: Juvenile dermatomyositis (JDM) is a rare and serious systemic autoimmune condition, and much remains unknown about the pathogenesis, the immune cell types and cell-specific…
Abstract Number: 008 • 2023 Pediatric Rheumatology Symposium
Treatment Response and Outcomes of 63 Cases of Juvenile Dermatomyositis-Associated Calcinosis
Belina Yi¹, Dawn Wahezi², Lauren Covert³, Kaveh Ardalan⁴, Joyce Hui-Yuen⁵, Natalia Vasquez Canizares², Doaa Mosad Mosa⁶, Madison Jones⁷, Colleen Correll⁸, Alexis Begezda⁹, Susan Shenoi¹⁰, Eveline Wu¹¹, Leonard Kovalick¹², William Lapin¹³, Stacey Tarvin¹⁴, Melissa Oliver¹⁵, Martha Rodriguez¹⁴, Itay Marmor¹⁶, Kevin Baszis¹⁷, Alysha Taxter¹⁸, Andrew Hanson¹⁹, Cynthia Crowson¹⁹ and Amir Orandi¹⁹, ¹Children's Hospital Los Angeles, Los Angeles, CA, ²Children's Hospital at Montefiore, New York, NY, ³Duke University, Durham, NC, ⁴Duke University School of Medicine, Durham, NC, ⁵Cohen Children's Medical Center, Northwell Health, Lake Success, New York; Center for Autoimmune, Musculoskeletal, and Hematopoietic Diseases Research, Feinstein Institutes for Medical Research, Northwell Health, Manhasset, NY, ⁶Mansoura University Hospitals, Mansoura University Faculty of Medicine, El Mansoura, Egypt, ⁷Keck School of Medicine at University of Southern California, Los Angeles, CCA, ⁸University of Minnesota, Minneapolis, MN, ⁹Penn State, State College, PA, ¹⁰Seattle Children's Hospital, Seattle, WA, ¹¹UNC Chapel Hill, Chapel Hill, NC, ¹²UNC Health Care, Durham, NC, ¹³Connecticut Children's, Hartford, CT, ¹⁴Riley Hospital for Children at Indiana University Health, Indianapolis, IN, ¹⁵Indiana University, Indianapolis, IN, ¹⁶Dana-Dwek Children's Hospital, Hod Hasharon, Israel, ¹⁷Washington University School of Medicine, St Louis, MO, ¹⁸Nationwide Children's Hospital, Columbus, OH, ¹⁹Mayo Clinic, Rochester, MN
Background/Purpose: Calcinosis is a poorly understood and morbid complication of juvenile dermatomyositis (JDM). As there is no consensus treatment approach for calcinosis, and limited knowledge…
Abstract Number: 018 • 2023 Pediatric Rheumatology Symposium
Using the Electronic Health Record to Identify Subjects with Rheumatic Disease
Alysha Taxter¹, Matthew Basiaga², Rajdeep Pooni³, Caitlan Pinotti⁴, Lisa Buckley⁵ and CARRA Registry Investigators⁶, ¹Nationwide Children's Hospital, Columbus, OH, ²Mayo Clinic, Rochester, MN, ³Stanford University, Palo Alto, CA, ⁴Duke, Durham, NC, ⁵Vanderbilt University Medical Center, Nashville, TN, ⁶CARRA, Washington, DC
Background/Purpose: Research teams spend hours manually searching the electronic health records (EHRs) to identify potential candidates eligible for recruitment to the Childhood Arthritis and Rheumatology…
Abstract Number: 023 • 2023 Pediatric Rheumatology Symposium
Effect of Type 1 Interferons and JAK Inhibitors on Gene Expression in Bioengineered Pediatric Skeletal Muscle
Lauren Covert¹, Joseph Prinz², Hailee Patel³, Jeffrey Dvergsten⁴ and George Truskey³, ¹Duke University, Durham, NC, ²Duke University School of Medicine, Department of Biostatistics and Bioinformatics, Durham, NC, ³Duke University, Department of Biomedical Engineering, Durham, NC, ⁴Duke University Hospital, Durham, NC
Background/Purpose: Genetic studies of new-onset juvenile dermatomyositis (JDM) exhibit elevation of Type 1 interferons (IFN 1) IFNα and IFNβ in blood, skin, and muscle. To…
Abstract Number: 027 • 2023 Pediatric Rheumatology Symposium
Identifying and Understanding JDM in Africa: A Survey of Rheumatology Care Providers from Africa
Jessica Perfetto¹, Laura Lewandowski², Dawn Wahezi¹, Christiaan Scott³ and Angela Migowa⁴, ¹Children's Hospital at Montefiore, New York, NY, ²NIAMS, NIH, Bethesda, MD, ³Red Cross War Memorial Children's Hospital, ⁴Aga Khan University, Nairobi, Kenya
Background/Purpose: There is a paucity of data on pediatric rheumatic disease (PRD) in low and middle-income countries (LMIC), creating a false perception of low prevalence…
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: 066 • 2023 Pediatric Rheumatology Symposium
Telemedicine Use in the Assessment of Juvenile Myositis: A Mixed-Methods Study of an International Healthcare Provider Experience
Y. Ingrid Goh¹, Peter Blier², Bianca Lang³, Marietta De Guzman⁴, Julie Fuller⁵, Kristin Houghton⁶, Kathryn Cook⁷, Susan Kim⁸, Vanessa Carbone¹, Heather Tory⁹, Jo-Anne Marcuz¹, Albert Chow¹⁰, Liza McCann¹¹, Charalampia Papadopoulou¹², Clarissa Pilkington¹³ and Stacey Tarvin¹⁴, ¹The Hospital for Sick Children, Toronto, ON, Canada, ²Retired, Amherst, MA, ³Dalhousie University - Halifax, Halifax, NS, Canada, ⁴Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ⁵UT Southwestern, Dallas, TX, ⁶University of British Columbia, Vancouver, BC, Canada, ⁷Akron Children's Hospital, Akron, OH, ⁸UCSF Benioff Children's Hospital, San Francisco, CA, ⁹Connecticut Children's Medical Center, S Glastonbury, CT, ¹⁰Loma Linda University, Loma Linda, CA, ¹¹Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, ¹²UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, ¹³Great Ormond Street Hospital, London, United Kingdom, ¹⁴Riley Hospital for Children at Indiana University Health, Indianapolis, IN
Background/Purpose: Care of patients with juvenile myositis (JM) involves complex assessments performed by specialized healthcare providers (HCPs). Restrictions during the COVID-19 pandemic required the rapid…
Abstract Number: 070 • 2023 Pediatric Rheumatology Symposium
The Impact of the COVID-19 Pandemic on Patients with Juvenile Idiopathic Inflammatory Myopathies
Dawn Wahezi¹, Dominique Jerome¹, Evin Rothschild¹, Jeffrey Dvergsten², Stacey Tarvin³, Susan Kim⁴ and Tamar Rubinstein¹, ¹Children's Hospital at Montefiore, New York, NY, ²Duke University Hospital, Durham, NC, ³Riley Hospital for Children at Indiana University Health, Indianapolis, IN, ⁴UCSF Benioff Children's Hospital, San Francisco, CA
Background/Purpose: Since the onset of the COVID-19 pandemic, there have been concerns regarding the risks of SARS-CoV-2 infection in patients with juvenile idiopathic inflammatory myopathies…

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