{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T08:19:06Z","timestamp":1790929146482,"version":"4.1.0"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,7,13]],"date-time":"2019-07-13T00:00:00Z","timestamp":1562976000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000960","name":"American College of Rheumatology Research and Education Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000960","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008741","name":"European League Against Rheumatism","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008741","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Macrophage activation syndrome (<jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>) is a life\u2010threatening complication of systemic juvenile idiopathic arthritis (<jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content>). Early diagnosis is critical. Classification criteria for <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> in <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content> perform less well in the setting of cytokine\u2010directed therapies. The goal herein was to explore a simple ratio of serum ferritin to the erythrocyte sedimentation rate (<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content>) for diagnosis of <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> in the setting of <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content>, and to assess ferritin alone as a screening tool for identifying <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> of multiple etiologies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data from a large international cohort of <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content> patients with and without <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>, and from hospitalized patients with systemic infection (<jats:styled-content style=\"fixed-case\">SI<\/jats:styled-content>), were assessed for the ferritin:<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content> ratio and ferritin alone to identify <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> among <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content> patients. Moreover, data from a smaller cohort of <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> patients associated with multiple etiologies and febrile hospitalized controls were explored. For both cohorts and controls, receiver operating characteristic curves (<jats:styled-content style=\"fixed-case\">ROC<\/jats:styled-content>s) for the ferritin:<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content> ratio and ferritin alone were constructed, and areas under the curves (<jats:styled-content style=\"fixed-case\">AUC<\/jats:styled-content>s) were calculated. The Youden index was used to determine the optimal ferritin:<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content> ratio and ferritin alone cut points for diagnosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A ferritin:<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content> ratio of 21.5 was 82% sensitive and 78% specific for diagnosing <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> versus active <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content> without <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>. Ferritin alone with a set sensitivity of 95% (screening tool) had an 89.3% specificity of identifying all\u2010cause <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> versus febrile hospitalized children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The ferritin:<jats:styled-content style=\"fixed-case\">ESR<\/jats:styled-content> ratio is a practical tool for diagnosing <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> among <jats:styled-content style=\"fixed-case\">sJIA<\/jats:styled-content> patients, and serum ferritin alone is a remarkable screening tool for identifying <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> among febrile hospitalized children.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr2.11048","type":"journal-article","created":{"date-parts":[[2019,7,13]],"date-time":"2019-07-13T04:43:32Z","timestamp":1562993012000},"page":"345-349","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":77,"title":["Ferritin to Erythrocyte Sedimentation Rate Ratio: Simple Measure to Identify Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis"],"prefix":"10.1002","volume":"1","author":[{"given":"Esraa M. A.","family":"Eloseily","sequence":"first","affiliation":[{"name":"Assiut University, Assiut, Egypt, and Pediatric Rheumatology University of Alabama at Birmingham"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Minoia","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5284-1587","authenticated-orcid":false,"given":"Courtney B.","family":"Crayne","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Beukelman","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9658-0385","authenticated-orcid":false,"given":"Angelo","family":"Ravelli","sequence":"additional","affiliation":[{"name":"Istituto Giannina Gaslini, Genoa, Italy and University of Genova Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2661-3086","authenticated-orcid":false,"given":"Randy Q.","family":"Cron","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,7,13]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/gene.2012.3"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1586\/1744666X.2015.1058159"},{"key":"e_1_2_6_4_1","first-page":"1133","article-title":"Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis","volume":"34","author":"Behrens EM","year":"2007","journal-title":"J Rheumatol."},{"key":"e_1_2_6_5_1","first-page":"21","article-title":"Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis","volume":"42","author":"Goel S","year":"2012","journal-title":"Ann Clin Lab Sci."},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39332"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23482"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23277"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38690"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121131"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.127"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.24883"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.12.016"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38769"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bor.0000381996.69261.71"}],"container-title":["ACR Open Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.11048","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr2.11048","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.11048","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T14:21:05Z","timestamp":1759414865000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr2.11048"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7,13]]},"references-count":15,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2019,8]]}},"alternative-id":["10.1002\/acr2.11048"],"URL":"https:\/\/doi.org\/10.1002\/acr2.11048","archive":["Portico"],"relation":{},"ISSN":["2578-5745","2578-5745"],"issn-type":[{"value":"2578-5745","type":"print"},{"value":"2578-5745","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,7,13]]},"assertion":[{"value":"2019-04-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-06-17","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-07-13","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}