Abstracts tagged "dermatomyositis"

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: 101 • 2023 Pediatric Rheumatology Symposium
Differences in Clinical and Patient-reported Outcomes in Juvenile Dermatomyositis by Race and Ethnicity
Susan Kim¹, Rebecca Olveda² and Jessica Neely², ¹UCSF Benioff Children's Hospital, San Francisco, CA, ²UCSF, San Francisco, CA
Background/Purpose: Previous studies in juvenile dermatomyositis (JDM) have shown that patients from minoritized ethnicities and those with lower family income are more likely to have…
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: 0173 • ACR Convergence 2022
Cutaneous Manifestations, Clinical Trials, Safety Efficacy and Safety of Lenabasum in the Phase 3 DeterMine Trial in Dermatomyositis
Victoria Werth¹, Barbara White², Josef Concha³, Josh Dan¹, Nancy Dgetluck², Kathleen Hally², Scott Constantine², Rohit Aggarwal⁴, David Fiorentino⁵, Ingrid Lundberg⁶ and Chester Oddis⁷, ¹Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, ²Corbus Pharmaceuticals, Norwood, MA, ³Universiity of Pennsylvania/Dermatology, Philadelphia, PA, ⁴University of Pittsburgh, Division of Rheumatology, Pittsburgh, PA, ⁵Stanford university/Department of Dermatology, Palo Alto, CA, ⁶Karolinska Institute, Stockholm, Sweden, ⁷University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Safe and effective treatments are of significant unmet need in DM. Lenabasum, a CB2 agonist that activates resolution of inflammation, improved skin disease, patient-reported…
Abstract Number: 1361 • ACR Convergence 2022
Factors Associated with Medication-Free Remission in Juvenile Dermatomyositis
Harneet Ghumman¹, Asra Firdous¹, Megan Quinlan-Waters¹, Amy Cassedy², Angela Merritt¹, Hermine Brunner³, Alexei Grom¹, Daniel Lovell¹ and Sheila Angeles-Han¹, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cleveland, OH, ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Department of Pediatrics, Cincinnati, OH
Background/Purpose: Juvenile dermatomyositis (JDM) is characterized by symmetric proximal muscle weakness, distinct rash, and a risk for calcinosis and multi-organ involvement. Treatment with systemic immunosuppression…
Abstract Number: 1860 • ACR Convergence 2022
Lenabasum, a Cannabinoid Type 2 Receptor Agonist, Activates Diverse Cell-specific Pathways in Whole Blood Leukocytes in Dermatomyositis
Nilesh Kodali¹, Thomas Vazquez², DeAnna Diaz³, Josh Dan⁴, Grant Sprow⁵, Rohan Dhiman⁶, Mariko Ogawa-Momohara⁷, Muhammad Bashir⁶, Meena Sharma⁸ and Victoria Werth⁹, ¹New Jersey Medical School, Coppell, TX, ²FIU Wertheim College of Medicine, Virginia Beach, VA, ³Philadelphia College of Medicine, Philadelphia, PA, ⁴Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, ⁵Albert Einstein College of Medicine, Philadelphia, PA, ⁶University of Pennsylvania, Philadelphia, PA, ⁷Nagoya University Graduate School of Medicine, Nagoya, Japan, ⁸Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ⁹University of Pennsylvania and Corporal Michael J. Crescenz VA Medical Center, Philadelphia, PA
Background/Purpose: Dermatomyositis (DM) is a systemic autoimmune disease that affects the skin and muscle. Lenabasum, a cannabinoid type 2 receptor (CB2R) agonist, has been developed…
Abstract Number: 0174 • ACR Convergence 2022
Intra-rater Validation of Calcinosis Durometer Measurements in Juvenile and Adult Dermatomyositis
Meghan Nelson¹, Adam Schiffenbauer², Hanna Kim³, Scott Gillespie⁴, Vy Do⁵, Julie Fuller⁶, Lisa G Rider⁷ and Kelly Rouster-Stevens⁸, ¹Division of Pediatric Rheumatology, Department of Pediatrics, Emory University School of Medicine; Children's Healthcare of Atlanta, Atlanta, GA, ²National Institutes of Health, Bethesda, MD, ³Division 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, ⁴Emory University, Atlanta, GA, ⁵UT Austin Dell Medical School, Austin, TX, ⁶UT Southwestern, Frisco, TX, ⁷Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD, ⁸Division of Pediatric Rheumatology, Department of Pediatrics, Emory University School of Medicine; Children’s Healthcare of Atlanta, Atlanta, GA
Background/Purpose: Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies primarily affecting proximal skeletal muscle and skin. Calcinosis, a known complication of DM, is…
Abstract Number: 1362 • ACR Convergence 2022
Validation of the 2016 ACR/EULAR Myositis Response Criteria in Juvenile Dermatomyositis (JDM) Clinical Trials and Consensus Profiles
Hanna Kim¹, Didem Saygin², Christian Douglas³, john mcgrath³, Jesse Wilkerson³, angela Pistorio⁴, Ann Reed⁵, Chester Oddis⁶, Frederick Miller⁷, Jiří Vencovský⁸, Nicola Ruperto⁹, Rohit Aggarwal¹⁰ and Lisa G Rider⁷, ¹Division 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, ²University of Chicago, Chicago, IL, ³Social and Scientific Systems, Inc., Durham, NC, ⁴IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁵Duke University School of Medicine, Durham, NC, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD, ⁸Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, ⁹IRCCS Istituto Giannina Gaslini; PRINTO, Clinica Pediatrica e Reumatologia, Genova, Italy, ¹⁰Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Juvenile dermatomyositis (JDM) ACR-EULAR myositis response criteria (MRC) were developed based on absolute % changes in 6 core set measures (CSM) differentially weighted to…
Abstract Number: 1861 • ACR Convergence 2022
Racial Disparities in Diagnosis and Treatment of Patients with Dermatomyositis of Different Skin Tones
Hillary Weisleder¹, 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: A lack of education in dermatopathology of non-white skin is of growing concern in the field of medical education. Diseases such as Dermatomyositis (DM)…
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: 1363 • ACR Convergence 2022
Impact of SARS-CoV-2 on the Clinical Presentation of Juvenile Idiopathic Inflammatory Myopathies
Jessica Perfetto¹, Donna Yoo², Carolina Tamashiro³, Megan Perron⁴, Natalia Vasquez Canizares⁵ and Dawn Wahezi⁶, ¹The Children's Hospital at Montefiore, Division of Rheumatology, Bronx, NY, ²Albert Einstein College of Medicine, Bronx, NY, ³The Children's Hospital at Montefiore, Department of Pediatrics, Bronx, NY, ⁴Children's Hospital Colorado, Division of Rheumatology, Aurora, CO, ⁵Children's Hospital at Montefiore/ Albert Einstein College of Medicine, New York, NY, ⁶Children's Hospital at Montefiore, Bronx, NY
Background/Purpose: Viruses can trigger juvenile idiopathic inflammatory myopathies (JIIM), including juvenile dermatomyositis (JDM), juvenile polymyositis (JPM), and overlap myositis. There is growing evidence that infection…
Abstract Number: 1865 • ACR Convergence 2022
Internet-based Enrollment for Myositis Studies Utilizing Patient Self-reported Diagnostic Criteria
Raisa Lomanto Silva¹, Silvia Martinez Laverde¹, Siamak Moghadam-Kia¹, Nicole Neiman², Dana Ascherman³, Chester Oddis³ and Rohit Aggarwal⁴, ¹University of Pittsburgh Medical Center, Pittsburgh, PA, ²Division of Rheumatology and Clinical Immunology, Department of Medicine, Pittsburgh, PA, ³University of Pittsburgh, Pittsburgh, PA, ⁴Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Recruitment is a major challenge in myositis clinical studies, which is hindered by the rarity and heterogeneity of the disease. Internet-based clinical studies using…
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: 1370 • ACR Convergence 2022
Abatacept in the Treatment of Refractory Juvenile Dermatomyositis
Gulnara Mamyrova¹, William Nguyen², Hassan Awal¹, Derek Jones¹, Alison Ehrlich³, Kathleen Brindle⁴, Shahriar Haji-Momenian⁴, Robert Sheets⁵, Adeline Y. Chin⁶, Shajia Lu⁷, Massimo Gadina⁷, Hanna Kim⁸, Olcay Y. Jones⁹, Lisa G Rider¹⁰ and Rodolfo V Curiel¹, ¹Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, DC, ²Division of Rheumatology, Department of Medicine, George Washington University, School of Medicine and Health Sciences, Washington, DC, ³Department of Dermatology, George Washington University School of Medicine and Health Sciences, Washington, DC, ⁴Department of Radiology, George Washington University School of Medicine and Health Sciences, Washington, DC, ⁵Division of Allergy, Immunology and Rheumatology, Department of Pediatrics, University of California San Diego, Rady Children's Hospital, San Diego, CA, ⁶Juvenile Myositis Therapeutic and Translation Studies Unit, PTRB, NIAMS, NIH, Bethesda, MD, ⁷Translational Immunology Section, NIAMS, NIH, Bethesda, MD, ⁸Division 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, ⁹Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences; Pediatric Rheumatology, Walter Reed National Military Medical Center, Bethesda, MD, ¹⁰Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences (NIEHS), National Institutes of Health, Bethesda, MD
Background/Purpose: This open-label 24-week study was conducted to evaluate the safety and efficacy of abatacept in patients with refractory juvenile dermatomyositis (JDM).Methods: Ten patients of…

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