{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T17:58:10Z","timestamp":1788976690581,"version":"build-2803163510"},"reference-count":35,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2023,6,7]],"date-time":"2023-06-07T00:00:00Z","timestamp":1686096000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007108","name":"George Washington University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001009","name":"BMSF","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001009","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100018208","name":"Cure JM Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100018208","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000066","name":"The National Institute of Environmental Health Sciences","doi-asserted-by":"publisher","award":["ES1010081"],"award-info":[{"award-number":["ES1010081"]}],"id":[{"id":"10.13039\/100000066","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"The National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["AR\u2010041215\u201002"],"award-info":[{"award-number":["AR\u2010041215\u201002"]}],"id":[{"id":"10.13039\/100000069","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":[[2023,7]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>This open\u2010label, 24\u2010week study was conducted to evaluate the safety and efficacy of abatacept in patients with refractory juvenile dermatomyositis (DM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Ten patients \u22657\u2009years of age with moderate disease activity were enrolled in a 24\u2010week study to examine the safety of subcutaneous abatacept and patient responses to the treatment. The primary endpoint was the International Myositis Assessment and Clinical Studies (IMACS) group Definition Of Improvement (DOI). Secondary endpoints included safety, changes in the core set activity measures (CSMs) of the IMACS group and the Pediatric Rheumatology International Trials Organization, and improvements in disease activity based on the American College of Rheumatology (ACR)\/EULAR response criteria for juvenile DM. Radiologists blinded with regard to participant data assessed magnetic resonance images (MRIs) of patient thigh muscles. Interferon (IFN)\u2013regulated gene score was performed on whole\u2010blood RNA samples using a NanoString assay, and cytokines were assessed using a Luminex assay.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Five patients achieved DOI at week 12, and 9 patients achieved DOI at week 24, including 2 patients with minimal, 4 patients with moderate, and 3 patients with major improvement by the 2016 ACR\/EULAR response criteria for juvenile DM when patients were assessed using the CSMs of the IMACS Group. Improvements from baseline were seen in all CSMs at weeks 12 and 24, except in muscle enzymes. Daily glucocorticoid doses decreased from a mean of 16.7 mg at baseline to 10.2 mg at week 24 (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.002). Average MRI muscle edema scores decreased from a mean baseline score of 5.3 to 2.3 at week 24 (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.01). Six patients had down\u2010trending IFN\u2010regulated gene scores and galectin\u20109 expression at week 24. Decreases in IFN\u2010regulated gene scores and in levels of interferon\u2010\u03b3\u2010inducible protein 10kDa, galectin\u20109, and interleukin\u20102 correlated with improvements in disease activity and in muscle edema shown on MRI. Eleven grade 2 or 3 treatment\u2010emergent adverse events were observed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This open\u2010label study demonstrated that abatacept may be beneficial for patients with treatment\u2010refractory juvenile DM.<\/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\/art42450-toc-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  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Medicine George Washington University School of Medicine and Health Sciences, Washington DC, and Juvenile Myositis Therapeutic and Translation Studies Unit, Pediatric Translation Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4975-1070","authenticated-orcid":false,"given":"Olcay Y.","family":"Jones","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Walter Reed National Military Medical Center, Bethesda, Maryland, and Division of Rheumatology, Department of Medicine, George Washington University School of Medicine and Health Sciences  Washington DC"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6912-2458","authenticated-orcid":false,"given":"Lisa G.","family":"Rider","sequence":"additional","affiliation":[{"name":"Environmental Autoimmunity Group, 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