{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T18:21:26Z","timestamp":1788373286035,"version":"build-2803163510"},"reference-count":40,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,8,12]],"date-time":"2022-08-12T00:00:00Z","timestamp":1660262400000},"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":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aims<\/jats:title><jats:p>We aim to perform ultrastructural and histopathological analysis of muscle biopsies from a large group of systemic sclerosis (SSc) patients, including some with early\/mild SSc features, and examine whether capillary pathology differentiates \u2018scleromyositis\u2019 (SM) from other auto\u2010immune myositis (AIM) subsets.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Muscle biopsies from a total of 60 SM patients and 43 AIM controls from two independent cohorts were examined by electron microscopy, collagen\u20104 immunofluorescence (Col4IF) and routine light microscopy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ultrastructural examination revealed prominent capillary basement membrane (BM) reduplication (4+ layers in &gt;50% of capillaries) in 65% of SM vs 0% of AIM controls (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). In SM cases without prominent BM reduplication, capillary dilation was the most distinctive feature, present in 8% of capillaries in SM vs 2% in controls (<jats:italic>p<\/jats:italic>\u2009=\u20090.001). Accumulation of ensheathed pericyte processes was another characteristic feature of SM and closely correlated with the degree of BM reduplication (<jats:italic>r<\/jats:italic>\u2009=\u20090.833, <jats:italic>p\u2009&lt;<\/jats:italic>\u00a00.001). On light microscopy, BM marker Col4IF revealed more frequent capillary enlargement in SM than in controls (84% vs 21%, <jats:italic>p\u2009&lt;<\/jats:italic>\u00a00.001). SM cases were classified as non\u2010inflammatory myopathy (36%), non\u2010specific myositis (33%) or immune\u2010mediated necrotizing myopathy (31%), but despite this histopathological heterogeneity, prominent BM reduplication remained a constant finding. In the 16 SM patients with early\/mild SSc features, 63% showed prominent BM reduplication.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These results show that capillary pathology, and in particular prominent capillary BM reduplication, is the hallmark histopathological feature of SM even in patients with early\/mild SSc and support the concept of SM as an organ manifestation of SSc and a distinct subset of AIM.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12840","type":"journal-article","created":{"date-parts":[[2022,7,27]],"date-time":"2022-07-27T13:09:15Z","timestamp":1658927355000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Capillary pathology with prominent basement membrane reduplication is the hallmark histopathological feature of scleromyositis"],"prefix":"10.1111","volume":"48","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8716-7924","authenticated-orcid":false,"given":"Benjamin","family":"Ellezam","sequence":"first","affiliation":[{"name":"Division of Pathology, CHU Sainte\u2010Justine, Department of Pathology and Cell Biology Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Leclair","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Jewish General Hospital, Department of Medicine McGill University  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"Troyanov","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, H\u00f4pital du Sacr\u00e9\u2010Coeur, Department of Medicine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Qu\u00e9bec 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Universitaire R\u00e9gional (CHAUR) du CIUSSS Mauricie Centre\u2010du\u2010Qu\u00e9bec, Department of Medicine Universit\u00e9 de Montr\u00e9al  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason","family":"Karamchandani","sequence":"additional","affiliation":[{"name":"Department of Pathology Montreal Neurological Institute and Hospital  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin","family":"O'Ferrall","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurosurgery and Department of Pathology McGill University and the Montreal Neurological Institute and Hospital  Montr\u00e9al Qu\u00e9bec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9atrice","family":"Lannes","sequence":"additional","affiliation":[{"name":"Service de Pathologie H\u00f4pitaux Universitaires de Strasbourg  Strasbourg 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