{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T03:56:01Z","timestamp":1790222161831,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,11,22]],"date-time":"2018-11-22T00:00:00Z","timestamp":1542844800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analysing different DM subforms, and to test the superiority of MxA to other markers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Immunohistochemistry for MxA and retinoic acid\u2010inducible gene I (RIG\u2010I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti\u2010Mi\u20102 (<jats:italic>n<\/jats:italic>\u00a0=\u00a06), \u2010transcription intermediary factor 1 gamma (<jats:italic>n<\/jats:italic>\u00a0=\u00a010), \u2010nuclear matrix protein 2 (<jats:italic>n<\/jats:italic>\u00a0=\u00a013), \u2010melanoma differentiation\u2010associated gene 5 (MDA5) (<jats:italic>n<\/jats:italic>\u00a0=\u00a010) or \u2010small ubiquitin\u2010like modifier activating enzyme (<jats:italic>n<\/jats:italic>\u00a0=\u00a01) autoantibodies and with no detectable autoantibody (<jats:italic>n<\/jats:italic>\u00a0=\u00a017). Among the patients, nine suffered from cancer and 22 were juvenile\u2010onset type. Disease controls included antisynthetase syndrome (ASS)\u2010associated myositis (<jats:italic>n<\/jats:italic>\u00a0=\u00a030), immune\u2010mediated necrotizing myopathy (<jats:italic>n<\/jats:italic>\u00a0=\u00a09) and inclusion body myositis (<jats:italic>n<\/jats:italic>\u00a0=\u00a05).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sarcoplasmic MxA expression featured 77% sensitivity and 100% specificity for overall DM patients, while RIG\u2010I staining and PFA reached respectively 14% and 59% sensitivity and 100% and 86% specificity. In any subset of DM, sarcoplasmic MxA expression showed higher sensitivity than RIG\u2010I and PFA. Some anti\u2010MDA5 antibody\u2010positive DM samples distinctively showed a scattered staining pattern of MxA. No ASS samples had sarcoplasmic MxA expression even though six patients had DM skin rash.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Sarcoplasmic MxA expression is more sensitive than PFA and RIG\u2010I expression for a pathological diagnosis of DM, regardless of the autoantibody\u2010related subgroup. In light of its high sensitivity and specificity, it may be considered a pathological hallmark of DM <jats:italic>per se<\/jats:italic>. Also, lack of MxA expression in ASS supports the idea that ASS is a distinct entity from DM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12519","type":"journal-article","created":{"date-parts":[[2018,9,29]],"date-time":"2018-09-29T00:39:14Z","timestamp":1538181554000},"page":"513-522","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":76,"title":["Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4758-183X","authenticated-orcid":false,"given":"A.","family":"Uruha","sequence":"first","affiliation":[{"name":"Mixed Research Unit (UMR) 974 Center of Research in Myology Institute of Myology Piti\u00e9\u2010Salp\u00eatri\u00e8re University Hospital National Institute of Health and Medical Research (INSERM) Paris\u2010Sorbonne University Paris 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