{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T01:19:22Z","timestamp":1792718362451,"version":"4.5.0"},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,3,24]],"date-time":"2021-03-24T00:00:00Z","timestamp":1616544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100015629","name":"Jerome L. Greene Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100015629","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5 K23 NS091379"],"award-info":[{"award-number":["5 K23 NS091379"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30\u2010AR070254"],"award-info":[{"award-number":["P30\u2010AR070254"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01GM129085"],"award-info":[{"award-number":["R01GM129085"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004319","name":"Pfizer","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004319","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":[[2021,5]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>This open\u2010label 12\u2010week study was conducted to evaluate the efficacy and safety of tofacitinib, a JAK inhibitor, in treatment\u2010refractory active dermatomyositis (DM).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Tofacitinib in extended\u2010release doses of 11 mg was administered daily to 10 subjects with DM. Prior to treatment, a complete washout of all steroid\u2010sparing agents was performed. The primary outcome measure was assessment of disease activity improvement based on the International Myositis Assessment and Clinical Studies group definition of improvement. Response rate was measured as the total improvement score according to the 2016 American College of Rheumatology (ACR)\/European League Against Rheumatism (EULAR) myositis response criteria. Secondary outcome measures included Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) scores, chemokine levels, immunohistochemical analysis of STAT1 expression in the skin, RNA sequencing analysis, and safety.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>At 12 weeks, the primary outcome was met in all 10 subjects. Five (50%) of 10 subjects experienced moderate improvement in disease activity, and the other 50% experienced minimal improvement according to the 2016 ACR\/EULAR myositis response criteria. The secondary outcome of the mean change in the CDASI activity score over 12 weeks was statistically significant (mean \u00b1 SD 28 \u00b1 15.4 at baseline versus 9.5 \u00b1 8.5 at 12 weeks) (<jats:italic>P<\/jats:italic> = 0.0005). Serum chemokine levels of CXCL9\/CXCL10 showed a statistically significant change from baseline. A marked decrease in STAT1 signaling in association with suppression of interferon target gene expression was demonstrated in 3 of 9 skin biopsy samples from subjects with dermatomyositis. The mean \u00b1 SD level of creatine kinase in the 10 subjects at baseline was 82 \u00b1 34.8 IU\/liter, highlighting that disease activity was predominantly located in the skin.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the first prospective, open\u2010label clinical trial of tofacitinib in DM that demonstrates strong clinical efficacy of a pan\u2010JAK inhibitor, as measured by validated myositis response criteria. Future randomized controlled trials using JAK inhibitors should be considered for treating DM.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.41602","type":"journal-article","created":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T10:57:18Z","timestamp":1606820238000},"page":"858-865","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":154,"title":["Study of Tofacitinib in Refractory Dermatomyositis: An Open\u2010Label Pilot Study of Ten Patients"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8436-1601","authenticated-orcid":false,"given":"Julie J.","family":"Paik","sequence":"first","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4172-1539","authenticated-orcid":false,"given":"Livia","family":"Casciola\u2010Rosen","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph Yusup","family":"Shin","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8030-9504","authenticated-orcid":false,"given":"Jemima","family":"Albayda","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4749-870X","authenticated-orcid":false,"given":"Eleni","family":"Tiniakou","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doris\u00a0G.","family":"Leung","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Gutierrez\u2010Alamillo","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie","family":"Perin","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liliana","family":"Florea","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corina","family":"Antonescu","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sherry G.","family":"Leung","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grazyna","family":"Purwin","sequence":"additional","affiliation":[{"name":"Johns Hopkins University School of Medicine  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Koenig","sequence":"additional","affiliation":[{"name":"Pfizer Inc.  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