{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T05:46:09Z","timestamp":1793857569993,"version":"4.6.1"},"reference-count":40,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,10,26]],"date-time":"2014-10-26T00:00:00Z","timestamp":1414281600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this multinational, multicenter study, pediatric rheumatologists and hemato\u2010oncologists entered patient data collected retrospectively into a web\u2010based database.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and\n                      <jats:sc>d<\/jats:sc>\n                      \u2010dimer levels were the sole laboratory biomarkers showing a percentage change of &gt;50% between the pre\u2010MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one\u2010third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study provides information on the clinical spectrum and current management of systemic JIA\u2013associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.38802","type":"journal-article","created":{"date-parts":[[2014,7,30]],"date-time":"2014-07-30T10:56:14Z","timestamp":1406717774000},"page":"3160-3169","source":"Crossref","is-referenced-by-count":388,"title":["Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients"],"prefix":"10.1002","volume":"66","author":[{"given":"Francesca","family":"Minoia","sequence":"first","affiliation":[{"name":"Istituto Giannina Gaslini Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergio","family":"Dav\u00ec","sequence":"additional","affiliation":[{"name":"Istituto Giannina Gaslini Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"AnnaCarin","family":"Horne","sequence":"additional","affiliation":[{"name":"Karolinska University Hospital Solna Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erkan","family":"Demirkaya","sequence":"additional","affiliation":[{"name":"Gulhane Military Medical Faculty Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Bovis","sequence":"additional","affiliation":[{"name":"Istituto Giannina Gaslini Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caifeng","family":"Li","sequence":"additional","affiliation":[{"name":"Beijing Children's Hospital Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Lehmberg","sequence":"additional","affiliation":[{"name":"University Medical Center Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila","family":"Weitzman","sequence":"additional","affiliation":[{"name":"Hospital for Sick Children, Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Insalaco","sequence":"additional","affiliation":[{"name":"Ospedale Pediatrico Bambino Ges\u00f9 Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carine","family":"Wouters","sequence":"additional","affiliation":[{"name":"University Hospital Gasthuisberg Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Shenoi","sequence":"additional","affiliation":[{"name":"Seattle Children's Hospital Seattle Washington"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graciela","family":"Espada","sequence":"additional","affiliation":[{"name":"Hospital de Ninos Ricardo Gutierrez Buenos Aires Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seza","family":"Ozen","sequence":"additional","affiliation":[{"name":"Hacettepe University Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Anton","sequence":"additional","affiliation":[{"name":"Hospital Sant Joan de D\u00e9u Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raju","family":"Khubchandani","sequence":"additional","affiliation":[{"name":"Jaslok Hospital and Research Centre Mumbai India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo","family":"Russo","sequence":"additional","affiliation":[{"name":"Hospital de Pediatria Juan P. 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