{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T16:30:29Z","timestamp":1791822629800,"version":"4.3.4"},"reference-count":50,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,1,30]],"date-time":"2018-01-30T00:00:00Z","timestamp":1517270400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100006260","name":"Rheumatology Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006260","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To assess performance of the 2016 macrophage activation syndrome (<jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>) classification criteria for patients with systemic juvenile idiopathic arthritis (<jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>) who develop <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> while treated with biologic medications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic literature review was performed to identify patients with <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> while being treated with interleukin (<jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>)\u20101 and <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20106 blocking agents. Clinical and laboratory information was compared to a large previously compiled historical cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eighteen publications were identified, and after removing duplicates, 35 patients treated with canakinumab and 49 patients with tocilizumab were available for analysis; 5 anakinra\u2010treated patients were excluded due to limited numbers. <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> classification criteria were less likely to classify tocilizumab\u2010treated patients as having <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> compared to the historical cohort or canakinumab\u2010treated patients (56.7%, 78.5%, and 84%, respectively; <jats:italic>P<\/jats:italic> &lt; 0.01). Patients who developed <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> while treated with canakinumab trended towards lower ferritin at <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> onset than the historical cohort (4,050 versus 5,353 ng\/ml; <jats:italic>P<\/jats:italic> = 0.18) but had no differences in other cardinal clinical or laboratory features. In comparison, patients who developed <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> while treated with tocilizumab were less likely febrile and had notably lower ferritin levels (1,152 versus 5,353 ng\/ml; <jats:italic>P<\/jats:italic> &lt; 0.001). Other features of <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> were more pronounced in patients treated with tocilizumab, including lower platelet counts, lower fibrinogen, and higher aspartate aminotransferase levels. Mortality rates for patients with <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> treated with tocilizumab or canakinumab were not significantly different from the historical cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These findings show substantial alterations in <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> features that may limit utility of defined criteria for diagnosis of systemic <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content> patients treated with biologic agents.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23277","type":"journal-article","created":{"date-parts":[[2017,5,12]],"date-time":"2017-05-12T17:22:43Z","timestamp":1494609763000},"page":"409-419","source":"Crossref","is-referenced-by-count":117,"title":["Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic 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