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Far surpassing the initial simplistic view of being responsible for energy generation, mitochondria have gathered attention regarding their role in inflammatory processes, being able to fuel autoimmunity, as shown by the presence of anti-mitochondrial antibodies (AMAs) in up to 10% of IIM patients. As cellular respiration takes place, mitochondrial metabolites might help to shape the pro-inflammatory milieu in affected muscle, beyond generating reactive oxygen species, which are well-recognized inducers of damage-associated molecular patterns. A series of mitochondrial components might facilitate the sterile activation of pro-inflammatory cells and the production of several cytokines responsible for enhancing auto-immune responses. Marked variation in the mitochondrial genome has also been reported in IIM patients. As such, we summarize key historical and recent advances linking aberrations and instabilities of mitochondrial DNA to impaired muscle function. Besides discussing mitochondrial dysfunction as an essential part of IIM development, we also highlight possible associations between presence of AMAs and a particular phenotype of IIM, with its own characteristic clinical and radiological pattern. Finally, we present promising treatment approaches targeting mitochondria, while briefly discussing experimental models for gaining deeper insight into the disease process, and ultimately leading to novel drug development.<\/jats:p>","DOI":"10.5041\/rmmj.10493","type":"journal-article","created":{"date-parts":[[2023,4,30]],"date-time":"2023-04-30T11:00:51Z","timestamp":1682852451000},"page":"e0006","source":"Crossref","is-referenced-by-count":24,"title":["The Emerging Role of Mitochondrial Dysfunction in the Pathogenesis of Idiopathic Inflammatory Myopathies"],"prefix":"10.5041","volume":"14","author":[{"name":"Division of Rheumatology, University of Washington, Seattle, WA, USA","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge Armando","family":"Gonzalez-Chapa","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina Barguil","family":"Mac\u00eado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Lood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2983","published-online":{"date-parts":[[2023,4,30]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","DOI":"10.1016\/j.euroecorev.2020.103391","article-title":"The role of R&D and knowledge spillovers in innovation and productivity","author":"Audretsch","year":"2020","unstructured":"Audretsch, D. 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