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EVs have been implicated in the pathogenesis of various autoimmune diseases, including dermatomyositis (DM), an inflammatory autoimmune disease characterized by distinct cutaneous manifestations, myopathy, and lung disease. We sought to review the role of EVs in DM and understand how they contribute to the pathogenesis and clinical characterization of the disease. We summarized the research progress on EVs in dermatomyositis based on recent publications. EV cargoes, such as double-stranded DNA, microRNA, and proteins, contribute to DM pathogenesis and mediate the proinflammatory response and cytokine release through signaling pathways such as the stimulator of interferon genes (STING) pathway. These nucleic acids and proteins have been proposed as disease-specific, stable biomarkers to monitor disease activity and responses to therapy. They also correlate with clinical parameters, inflammatory markers, and disease severity scores. Furthermore, some markers show an association with morbidities of DM, such as muscle weakness and interstitial lung disease. The continued study of EVs will help us to further elucidate our understanding of dermatomyositis.<\/jats:p>","DOI":"10.3390\/ijms25041967","type":"journal-article","created":{"date-parts":[[2024,2,6]],"date-time":"2024-02-06T05:36:43Z","timestamp":1707197803000},"page":"1967","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Extracellular Vesicles in the Pathogenesis, Clinical Characterization, and Management of Dermatomyositis: A Narrative Review"],"prefix":"10.3390","volume":"25","author":[{"given":"Cristina","family":"Ricco","sequence":"first","affiliation":[{"name":"Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA 19104, USA"},{"name":"Department of Dermatology, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-6699-5956","authenticated-orcid":false,"given":"Ahmed","family":"Eldaboush","sequence":"additional","affiliation":[{"name":"Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA 19104, USA"},{"name":"Department of Dermatology, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8827-2024","authenticated-orcid":false,"given":"Ming-Lin","family":"Liu","sequence":"additional","affiliation":[{"name":"Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA 19104, USA"},{"name":"Department of Dermatology, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victoria P.","family":"Werth","sequence":"additional","affiliation":[{"name":"Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, PA 19104, USA"},{"name":"Department of Dermatology, School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/bs.acc.2016.06.005","article-title":"Microvesicles in autoimmune diseases","volume":"77","author":"Liu","year":"2016","journal-title":"Adv. Clin. 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