{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T10:45:39Z","timestamp":1767091539097,"version":"build-2065373602"},"reference-count":13,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2018,7,5]],"date-time":"2018-07-05T00:00:00Z","timestamp":1530748800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Research on Rare and Intractable Diseases, Health, and Labor Sciences"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2018,9]]},"abstract":"Objective<\/jats:title>To validate whether the 2016 American College of Rheumatology\/European League Against Rheumatism classification criteria of macrophage activation syndrome (MAS<\/jats:styled-content>) complicating systemic juvenile idiopathic arthritis (JIA<\/jats:styled-content>) is practical in the real world.<\/jats:p><\/jats:sec>Methods<\/jats:title>A combination of expert consensus and analysis of real patient data was conducted by a panel of 15 pediatric rheumatologists. A total of 65 profiles comprised 18 patients with systemic JIA<\/jats:styled-content>\u2013associated MAS<\/jats:styled-content> and 47 patients with active systemic JIA<\/jats:styled-content> without evidence of MAS<\/jats:styled-content>. From these profiles, 10 patient data points for full\u2010blown MAS<\/jats:styled-content>, 11 patient data points for MAS<\/jats:styled-content> onset, and 47 patient data points for acute systemic JIA<\/jats:styled-content> without MAS<\/jats:styled-content> were evaluated.<\/jats:p><\/jats:sec>Results<\/jats:title>Evaluation of the classification criteria to discriminate full\u2010blown MAS<\/jats:styled-content> from acute systemic JIA<\/jats:styled-content> without MAS<\/jats:styled-content> showed a sensitivity of 1.000 and specificity of 1.000 at the time of full\u2010blown MAS<\/jats:styled-content>. Sensitivity was 0.636 and specificity was 1.000 at the time of MAS<\/jats:styled-content> onset. The number of measurement items that fulfilled the criteria increased in full\u2010blown MAS<\/jats:styled-content> compared to that at MAS<\/jats:styled-content> onset. At MAS<\/jats:styled-content> onset, the positive rates of patients who met the criteria for platelet counts and triglycerides were low, whereas those for aspartate aminotransferase were relatively high. At full\u2010blown MAS<\/jats:styled-content>, the number of patients who met the criteria for each measurement item increased.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>The classification criteria for MAS<\/jats:styled-content> complicating systemic JIA<\/jats:styled-content> had a very high diagnostic performance. However, the diagnostic sensitivity for MAS<\/jats:styled-content> onset was relatively low. For the early diagnosis of MAS<\/jats:styled-content> in systemic JIA<\/jats:styled-content>, the dynamics of laboratory values during the course of MAS<\/jats:styled-content> should be further investigated.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23482","type":"journal-article","created":{"date-parts":[[2017,12,1]],"date-time":"2017-12-01T15:07:09Z","timestamp":1512140829000},"page":"1412-1415","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Validation of Classification Criteria of Macrophage Activation Syndrome in Japanese Patients With Systemic Juvenile Idiopathic Arthritis"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1077-7772","authenticated-orcid":false,"given":"Masaki","family":"Shimizu","sequence":"first","affiliation":[{"name":"Graduate School of Medical Sciences Kanazawa University Kanazawa 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