{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T18:50:08Z","timestamp":1791312608071,"version":"4.3.1"},"reference-count":20,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,1,29]],"date-time":"2025-01-29T00:00:00Z","timestamp":1738108800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Mixed connective tissue disease (MCTD) patients often have myositis, however, myopathological and clinical data for MCTD are limited. Recent reports have shown that the pathology of MCTD myositis resembles that of immune\u2010mediated necrotizing myopathy (IMNM), whereas earlier reports described perifascicular atrophy or inflammatory infiltrates predominantly in the perivascular region in MCTD myositis. We aim to describe the clinical and myopathological features of MCTD myositis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We analyzed the clinical and myopathological findings of nine myositis patients with U1\u2010RNP antibodies who fulfilled the diagnostic criteria for MCTD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eight patients had muscle weakness in the proximal extremities, and overall, six patients had atypical weakness in the face, neck, wrist, or fingers. Four of those patients required additional intensive treatment (intravenous immunoglobulin or methylprednisolone). Therapeutic responses were consistently favorable overall, and there were no deaths during the observation period. In biopsied muscle specimens, common findings were mild myogenic change, increased necrotic and regenerating fibers, and inflammatory infiltrates predominating in the perivascular region. Two specimens were classified into the spectrum of dermatomyositis (DM); the remaining seven specimens, which had a smaller number of necrotic fibers and nonspecific infiltration, were unclassifiable.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Patients with MCTD myositis often exhibit an axial or atypical distribution of muscle weakness, which may require intensive therapy. Histological study demonstrates the heterogeneity of myopathology of MCTD myositis and suggests that DM and underlying vasculopathy might be present in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28360","type":"journal-article","created":{"date-parts":[[2025,1,29]],"date-time":"2025-01-29T09:19:26Z","timestamp":1738142366000},"page":"583-592","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinicopathological Features of Mixed Connective Tissue Disease\u2010Related Myositis: A Case Series"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-7256-2308","authenticated-orcid":false,"given":"Naohiro","family":"Sakamoto","sequence":"first","affiliation":[{"name":"Department of Neurology Tohoku University Graduate School of Medicine  Sendai Japan"},{"name":"Department of Neurology, Anti\u2010Aging and Vascular Medicine, Division of Internal Medicine National Defense Medical College  Tokorozawa Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rumiko","family":"Izumi","sequence":"additional","affiliation":[{"name":"Department of Neurology Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Neurology Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maki","family":"Tateyama","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Iwate Hospital  Ichinoseki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masashi","family":"Aoki","sequence":"additional","affiliation":[{"name":"Department of Neurology Tohoku University Graduate School of Medicine  Sendai Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,29]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keac703"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(199905)42:5<899::AID-ANR8>3.0.CO;2-L"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1590\/S0004-282X2004000600001"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000007188"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102465"},{"key":"e_1_2_11_7_1","first-page":"41","volume-title":"Mixed Connective Tissue Disease and Antinuclear Antibodies","author":"Kasukawa R.","year":"1987"},{"key":"e_1_2_11_8_1","first-page":"33","volume-title":"Mixed Connective Tissue Disease and Anti\u2010Nuclear Antibodies","author":"Alarcon\u2010Segovia D.","year":"1987"},{"key":"e_1_2_11_9_1","first-page":"545","volume-title":"Les Maladies Systemiques","author":"Kahn M. 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