{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T08:53:04Z","timestamp":1784364784289,"version":"3.55.0"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,10,25]],"date-time":"2019-10-25T00:00:00Z","timestamp":1571961600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002855","name":"Ministry of Science and Technology of the People's Republic of China","doi-asserted-by":"publisher","award":["2016YFC1300605"],"award-info":[{"award-number":["2016YFC1300605"]}],"id":[{"id":"10.13039\/501100002855","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Dermatomyositis (DM) with anti\u2010nuclear matrix protein\u20102 (NXP\u20102) antibodies usually shows multifocal ischaemic lesions in muscle. Here, we aimed to investigate the microarteriopathy underlying muscle ischaemia in anti\u2010NXP\u20102\u2010positive DM.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 16 patients diagnosed with anti\u2010NXP\u20102\u2010positive DM were investigated by muscle biopsy. A total of 13 patients with DM with other myositis\u2010specific antibodies and 11 normal controls were included for comparison. Immunofluorescence assays were performed to localize endothelial cells, smooth muscle cells and pericytes, and to determine lesions in myofibers and microvessels by vascular endothelial growth factor and myxovirus resistance protein A (MxA). Electron microscopy was carried out to assess ultrastructure alterations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Subcutaneous edema, severe muscle weakness and dysphagia together with elevated creatine kinase, D\u2010dimer and triglyceride levels, and decreased albumin levels were found in anti\u2010NXP\u20102\u2010positive DM. Muscle ischaemia included regional muscle edema, perifascicular atrophy, microinfarcts and focal punched\u2010out vacuoles. The density of arterioles was higher in anti\u2010NXP\u20102\u2010positive DM (<jats:italic>P\u00a0<\/jats:italic>\uff1c\u00a00.05). Perimysial arterioles with thickened vascular wall, thrombosis and lipid accumulation were found in the vascular wall of diseased perimysial arterioles. The frequency of diseased arterioles and thrombosis was higher in anti\u2010NXP\u20102\u2010positive DM (<jats:italic>P\u00a0<\/jats:italic>&lt;\u00a00.05). Sarcoplasmic vascular endothelial growth factor and MxA expression was observed in multifocal ischaemic lesions. MxA was present in endothelial and smooth muscle cells of the diseased arterioles and pericytes. Electron microscopy confirmed damaged capillaries and tubuloreticular structures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our research suggested that perimysial arterioles were most commonly involved in anti\u2010NXP\u20102\u2010positive DM, which led to muscle ischaemia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14097","type":"journal-article","created":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T06:05:13Z","timestamp":1569909913000},"page":"514-521","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Perimysial microarteriopathy in dermatomyositis with anti\u2010nuclear matrix protein\u20102 antibodies"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8908-6194","authenticated-orcid":false,"given":"Y.","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Neurology Peking University First Hospital Beijing 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