{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T22:09:12Z","timestamp":1791929352788,"version":"4.3.4"},"reference-count":53,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T00:00:00Z","timestamp":1694995200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009592","name":"Beijing Municipal Science and Technology Commission","doi-asserted-by":"publisher","award":["Z191100006619034"],"award-info":[{"award-number":["Z191100006619034"]}],"id":[{"id":"10.13039\/501100009592","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Anti\u2010mitochondrial antibodies (AMAs) are associated with idiopathic inflammatory myopathies (IIMs). We aimed to summarize the clinicopathological characteristics, assess circulating cell\u2010free mitochondrial DNA (ccf\u2010mtDNA), and circulating cell\u2010free nuclear DNA (ccf\u2010nDNA) in AMA\u2010associated IIMs.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Medical records of 37 IIMs patients with AMAs were reviewed. Circulating cell\u2010free mtDNA and ccf\u2010nDNA levels in sera from IIMs patients with AMAs (<jats:italic>n<\/jats:italic>\u2009=\u200921), disease controls (<jats:italic>n<\/jats:italic>\u2009=\u200966) and healthy controls (HCs) (<jats:italic>n<\/jats:italic>\u2009=\u200923) were measured and compared. Twenty\u2010eight immune\u2010mediated necrotizing myopathy (IMNM) patients, 23 dermatomyositis (DM) patients, and 15 anti\u2010synthetase syndrome (ASS) patients were enrolled as disease controls. Correlations between variables were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Limb weakness was observed in 75.7% and neck weakness in 56.8% of patients. Cardiac involvement occurred in 51.4% of patients. Muscle pathology revealed 81.1% of IMNM, 5.4% polymyositis, and 13.5% nonspecific myositis. Microinfarction was observed in 8.1% of patients. Serum ccf\u2010mtDNA levels in AMA\u2010associated IIMs were significantly higher than those in HCs (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), but no significant differences between AMA\u2010associated IIMs and IMNM, DM, or ASS. Serum ccf\u2010nDNA levels in AMA\u2010associated IIMs were significantly higher than those in HCs (<jats:italic>p<\/jats:italic>\u2009=\u20090.02), and significantly lower than those in DM (<jats:italic>p<\/jats:italic>\u2009=\u20090.02). Serum ccf\u2010nDNA levels correlated negatively with MMT8 total scores (<jats:italic>r<\/jats:italic>s\u2009=\u2009\u22120.458, <jats:italic>p<\/jats:italic>\u2009=\u20090.037) and positively with mRS scores (<jats:italic>r<\/jats:italic>s\u2009=\u20090.486, <jats:italic>p<\/jats:italic>\u2009=\u20090.025). Serum ccf\u2010nDNA levels were significantly higher in the non\u2010remission group (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>AMA\u2010associated IIMs exhibit distinct clinicopathological features. Serum ccf\u2010nDNA may serve as a potential marker for disease severity and prognosis in AMA\u2010associated IIMs.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51901","type":"journal-article","created":{"date-parts":[[2023,9,19]],"date-time":"2023-09-19T05:44:20Z","timestamp":1695102260000},"page":"2127-2138","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Clinicopathological and circulating cell\u2010free <scp>DNA<\/scp> profile in myositis associated with anti\u2010mitochondrial antibody"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6910-716X","authenticated-orcid":false,"given":"Yikang","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurology Peking University First Hospital  Beijing 100034 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