{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T04:51:24Z","timestamp":1790743884527,"version":"4.1.0"},"reference-count":41,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,5,2]],"date-time":"2021-05-02T00:00:00Z","timestamp":1619913600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["K01\u2010AR072129"],"award-info":[{"award-number":["K01\u2010AR072129"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["K24\u2010AR076975"],"award-info":[{"award-number":["K24\u2010AR076975"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["P30\u2010AR075043"],"award-info":[{"award-number":["P30\u2010AR075043"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["R01\u2010AR071384"],"award-info":[{"award-number":["R01\u2010AR071384"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008269","name":"Michigan Institute for Clinical and Health Research","doi-asserted-by":"publisher","award":["5 UL1 TR002240\u201004"],"award-info":[{"award-number":["5 UL1 TR002240\u201004"]}],"id":[{"id":"10.13039\/100008269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","award":["K12 HD028820\u201028"],"award-info":[{"award-number":["K12 HD028820\u201028"]}],"id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006260","name":"Rheumatology Research Foundation","doi-asserted-by":"publisher","award":["AWD014132"],"award-info":[{"award-number":["AWD014132"]}],"id":[{"id":"10.13039\/100006260","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2021,6]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Skin inflammation heralds systemic disease in juvenile myositis, yet we lack an understanding of pathogenic mechanisms driving skin inflammation in this disease. We undertook this study to define cutaneous gene expression signatures in juvenile myositis and identify key genes and pathways that differentiate skin disease in juvenile myositis from childhood\u2010onset systemic lupus erythematosus (SLE).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We used formalin\u2010fixed paraffin\u2010embedded skin biopsy samples from 15 patients with juvenile myositis (9 lesional, 6 nonlesional), 5 patients with childhood\u2010onset SLE, and 8 controls to perform transcriptomic analysis and identify significantly differentially expressed genes (DEGs; q \u2264 5%) between patient groups. We used Ingenuity Pathway Analysis (IPA) to highlight enriched biologic pathways and validated DEGs by immunohistochemistry and quantitative real\u2010time polymerase chain reaction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Comparison of lesional juvenile myositis to control samples revealed 221 DEGs, with the majority of up\u2010regulated genes representing interferon (IFN)\u2013stimulated genes.\n                      <jats:italic>CXCL10<\/jats:italic>\n                      ,\n                      <jats:italic>CXCL9<\/jats:italic>\n                      , and\n                      <jats:italic>IFI44L<\/jats:italic>\n                      represented the top 3 DEGs (fold change 23.2, 13.3, and 13.0, respectively; q &lt; 0.0001). IPA revealed IFN signaling as the top canonical pathway. When compared to childhood\u2010onset SLE, lesional juvenile myositis skin shared a similar gene expression pattern, with only 28 unique DEGs, including\n                      <jats:italic>FBLN2<\/jats:italic>\n                      ,\n                      <jats:italic>CHKA<\/jats:italic>\n                      , and\n                      <jats:italic>SLURP1<\/jats:italic>\n                      . Notably, patients with juvenile myositis who were positive for nuclear matrix protein 2 (NXP\u20102) autoantibodies exhibited the strongest IFN signature and also demonstrated the most extensive Mx\u20101 immunostaining, both in keratinocytes and perivascular regions.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Lesional juvenile myositis skin demonstrates a striking IFN signature similar to that previously reported in juvenile myositis muscle and peripheral blood. Further investigation into the association of a higher IFN score with NXP\u20102 autoantibodies may provide insight into disease endotypes and pathogenesis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.41615","type":"journal-article","created":{"date-parts":[[2020,12,11]],"date-time":"2020-12-11T01:56:31Z","timestamp":1607651791000},"page":"1062-1072","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Comparison of Lesional Juvenile Myositis and Lupus Skin Reveals Overlapping Yet Unique Disease Pathophysiology"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1127-7879","authenticated-orcid":false,"given":"Jessica L.","family":"Turnier","sequence":"first","affiliation":[{"name":"University of Michigan  Ann Arbor"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren 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