{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T11:30:23Z","timestamp":1792150223772,"version":"4.4.0"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,4,30]],"date-time":"2019-04-30T00:00:00Z","timestamp":1556582400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis & Rheumatology"],"published-print":{"date-parts":[[2019,6]]},"abstract":"Objective<\/jats:title>To evaluate the expression of type I interferon (IFN<\/jats:styled-content>\u03b1\/\u03b2)\u2013 and type II IFN<\/jats:styled-content> (IFN<\/jats:styled-content>\u03b3)\u2013inducible genes in muscle biopsy specimens from patients with juvenile dermatomyositis (DM<\/jats:styled-content>) and to correlate their expression levels with histologic and clinical features.<\/jats:p><\/jats:sec>Methods<\/jats:title>Expression levels of IFN<\/jats:styled-content>\u2010inducible genes and proinflammatory cytokines were assessed by quantitative polymerase chain reaction in muscle biopsy specimens from patients with juvenile DM<\/jats:styled-content> (n = 39), patients with Duchenne's muscular dystrophy (DMD<\/jats:styled-content>), and healthy controls. Muscle biopsy sections were stained and scored for severity of histopathologic features. The charts of patients with juvenile DM<\/jats:styled-content> were reviewed for clinical features at the time of sampling and long\u2010term outcomes.<\/jats:p><\/jats:sec>Results<\/jats:title>Muscle expression levels of IFN<\/jats:styled-content>\u03b1\/\u03b2\u2010inducible genes (type I IFN<\/jats:styled-content> score), IFN<\/jats:styled-content>\u03b3, IFN<\/jats:styled-content>\u03b3\u2010inducible genes (type II IFN<\/jats:styled-content> score), and tumor necrosis factor (TNF<\/jats:styled-content>) were significantly higher in juvenile DM patients not receiving glucocorticoid therapy before muscle biopsy (n = 27) compared to DMD<\/jats:styled-content> patients (n = 24) (type I IFN<\/jats:styled-content> score, P<\/jats:italic> < 0.0001; type II IFN<\/jats:styled-content> score, P<\/jats:italic> < 0.001; TNF, P<\/jats:italic> < 0.05) and healthy controls (n = 4) (type I IFN<\/jats:styled-content> score, P<\/jats:italic> < 0.01; type II IFN<\/jats:styled-content> score, P<\/jats:italic>\u00a0<\u00a00.01; TNF, P<\/jats:italic> < 0.05). Immunofluorescence staining of muscle biopsy sections from untreated juvenile DM patients showed increased immunoreactivity for IFN<\/jats:styled-content>\u03b3 and HLA<\/jats:styled-content> class II molecules compared to controls. Type I and type II IFN<\/jats:styled-content> scores were correlated with typical histopathologic features of juvenile DM muscle biopsy samples, such as infiltration of endomysial CD3+ cells (type I IFN<\/jats:styled-content> score, r = 0.68; type II IFN<\/jats:styled-content> score, r = 0.63), perimysial CD3+ cells (type I IFN<\/jats:styled-content> score, r = 0.59; type II IFN<\/jats:styled-content> score, r = 0.66), CD68+ cells (type II IFN<\/jats:styled-content> score, r = 0.46), and perifascicular atrophy (type I IFN<\/jats:styled-content> score, r = 0.61; type II IFN<\/jats:styled-content> score, r = 0.77). Juvenile DM patients with a high type I IFN<\/jats:styled-content> score, a high type II IFN<\/jats:styled-content> score, and high TNF expression levels showed more severe disease activity at biopsy (P<\/jats:italic> < 0.05). In addition, juvenile DM patients with a high type II IFN<\/jats:styled-content> score at biopsy reached clinically inactive disease significantly later than patients with low type II IFN<\/jats:styled-content> score (log rank chi\u2010square value 13.53, P<\/jats:italic> < 0.001).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>The increased expression of IFN<\/jats:styled-content>\u2010inducible genes in the muscle in juvenile DM<\/jats:styled-content> patients and their association with histologic and clinical features further support a pathogenic role for both type I and type II IFN<\/jats:styled-content>s in juvenile DM<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40800","type":"journal-article","created":{"date-parts":[[2018,12,15]],"date-time":"2018-12-15T09:43:37Z","timestamp":1544867017000},"page":"1011-1021","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":95,"title":["Muscle Expression of Type I and Type II<\/scp> Interferons Is Increased in Juvenile Dermatomyositis and Related to Clinical and Histologic Features"],"prefix":"10.1002","volume":"71","author":[{"given":"Gian Marco","family":"Moneta","sequence":"first","affiliation":[{"name":"Ospedale Pediatrico Bambino Ges\u00f9 IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Denise","family":"Pires Marafon","sequence":"additional","affiliation":[{"name":"Ospedale Pediatrico Bambino Ges\u00f9 IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5346-3357","authenticated-orcid":false,"given":"Emiliano","family":"Marasco","sequence":"additional","affiliation":[{"name":"Ospedale Pediatrico Bambino Ges\u00f9 IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Rosina","sequence":"additional","affiliation":[{"name":"University of Genoa Genoa 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