{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T02:09:55Z","timestamp":1791684595302,"version":"4.3.4"},"reference-count":149,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T00:00:00Z","timestamp":1743552000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003593","name":"National Council for Scientific and Technological Development","doi-asserted-by":"publisher","award":["305981\/2023-4"],"award-info":[{"award-number":["305981\/2023-4"]}],"id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003593","name":"National Council for Scientific and Technological Development","doi-asserted-by":"publisher","award":["301500\/2022-3"],"award-info":[{"award-number":["301500\/2022-3"]}],"id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003593","name":"National Council for Scientific and Technological Development","doi-asserted-by":"publisher","award":["001"],"award-info":[{"award-number":["001"]}],"id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior\u2014Brasil (CAPES)","doi-asserted-by":"publisher","award":["305981\/2023-4"],"award-info":[{"award-number":["305981\/2023-4"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior\u2014Brasil (CAPES)","doi-asserted-by":"publisher","award":["301500\/2022-3"],"award-info":[{"award-number":["301500\/2022-3"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior\u2014Brasil (CAPES)","doi-asserted-by":"publisher","award":["001"],"award-info":[{"award-number":["001"]}],"id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Idiopathic inflammatory myopathies are rare and complex representatives of systemic connective tissue diseases. Described initially as only two entities, recent advances in molecular and imaging techniques now divide them into many subtypes, each with unique pathogenesis and clinical phenotypes. Dermatomyositis and its juvenile form are the most prevalent subtypes and are characterized by systemic vasculopathy and humoral autoimmunity. Genetic predisposition and environmental triggers initiate immune tolerance breakdown, leading to autoantibody production, complement activation, and tissue damage. Anti-synthetase syndrome primarily affects the lungs, where immune responses to aminoacyl-RNA synthetases drive vasculopathy, lung inflammation, and fibrosis. Immune-mediated necrotizing myopathies are muscle-specific, with autoantibodies inducing fiber necrosis and atrophy. Lastly, sporadic inclusion body myositis is a slowly progressive myopathy in which dysfunctional protein handling and autophagy are more important pathogenic elements than muscle inflammation itself. The expanding body of basic science evidence can be overwhelming, making it challenging to connect pathogenic mechanisms to clinical manifestations. This review aims to address this challenge by presenting recent insights into myositis pathogenesis from a practical perspective, reinforcing the links between basic science and clinical semiology.<\/jats:p>","DOI":"10.3390\/ijms26073302","type":"journal-article","created":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T07:47:44Z","timestamp":1743580064000},"page":"3302","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Idiopathic Inflammatory Myopathies: Recent Evidence Linking Pathogenesis and Clinical Features"],"prefix":"10.3390","volume":"26","author":[{"given":"Eunice Fragoso","family":"Martins","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-881, Brazil"},{"name":"Post-Graduate Program in Medical Sciences, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-887, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla Helena","family":"Cappello","sequence":"additional","affiliation":[{"name":"Department of Orthopedics, Rheumatology and Traumatology, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-887, Brazil"},{"name":"Post-Graduate Program in Child and Adolescent Health, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-888, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel Katsuyuki","family":"Shinjo","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de S\u00e3o Paulo, S\u00e3o Paulo 01246-903, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Appenzeller","sequence":"additional","affiliation":[{"name":"Department of Orthopedics, Rheumatology and Traumatology, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-887, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3051-7663","authenticated-orcid":false,"given":"Jean Marcos","family":"de Souza","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, School of Medical Sciences, Universidade Estadual de Campinas (UNICAMP), Campinas 13083-881, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1038\/s41572-021-00321-x","article-title":"Idiopathic inflammatory myopathies","volume":"7","author":"Lundberg","year":"2021","journal-title":"Nat. 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