{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T10:39:17Z","timestamp":1792665557897,"version":"4.5.0"},"reference-count":19,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T00:00:00Z","timestamp":1786665600000},"content-version":"vor","delay-in-days":13,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Calcinosis cutis (CC) is dystrophic calcification affecting 20% to 40% of patients with juvenile dermatomyositis (JDM). Management of CC is often challenging owing to its being refractory to usual therapeutic options. Type I interferon (IFN)\u2010mediated immune dysregulation may be involved in the pathogenic role in CC, suggesting that altering JAK\/STAT signaling through JAK inhibitors might offer a therapeutic benefit.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this open\u2010label, single\u2010arm study, the primary objective was to study the effect of tofacitinib (MSN Pharmaceuticals) on the burden of CC in children (2\u201318 years) with JDM, as assessed by the Agatston score using a low\u2010dose whole\u2010body computed tomography scan at 24 \u00b1 2 weeks follow\u2010up. Secondary objectives were to evaluate the effect on Cutaneous Dermatomyositis Disease Area and Severity Index, Childhood Myositis and Assessment scales, steroid usage, IFN\u03b1 and IFN\u03b2 levels and adverse events. Children with JDM and clinical or radiologic evidence of CC were enrolled for this study. The study drug tofacitinib was administered orally as per the standard doses based on the participant's weight band. The standard therapy included concomitant steroids, methotrexate and\/or mycophenolate mofetil, and\/or intravenous immunoglobulin. Those who received cyclophosphamide or rituximab in the preceding six months, were receiving concomitant topical tacrolimus, had active tuberculosis, or had hematologic abnormalities were excluded.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Twenty children (11 boys) with a mean age of 10.4 (SD 3.6 years) completed the study. Eighteen children had CC associated with JDM, whereas two had JDM\u2010systemic sclerosis overlap. Median duration of CC in the study population was 31 (interquartile range [IQR] 19.5\u201354) months. There was a significant reduction in the Agatston score, median at follow\u2010up compared to baseline 6,349 (IQR 3,765\u201365,255) versus 4,007 (1,616\u201355,515),\n                      <jats:italic>P<\/jats:italic>\n                      = 0.012, with a moderate effect size (d\u2010robust \u22120.52). No serious adverse events were noted during the study period.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Tofacitinib, when added to standard therapy, reduced the Agatston score in children with CC associated with JDM. No major safety signals were observed during the study period.<\/jats:p>\n                    <jats:p>Clinical Trials Registry \u2010 India: CTRI\/2024\/02\/062740.<\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/acr290117-gra-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acr2.90117","type":"journal-article","created":{"date-parts":[[2026,8,15]],"date-time":"2026-08-15T06:15:47Z","timestamp":1786774547000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Tofacitinib for Calcinosis Cutis Associated With Juvenile Dermatomyositis: An Open\u2010Label Single\u2010Arm Study"],"prefix":"10.1002","volume":"8","author":[{"given":"Pragati","family":"Jain","sequence":"first","affiliation":[{"name":"Department of Pediatrics All India Institute of Medical Sciences  New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Supraja Laguduva","family":"Mohan","sequence":"additional","affiliation":[{"name":"Department of Radiodiagnosis and Interventional Radiology All India Institute of Medical Sciences  New 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