{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T02:54:45Z","timestamp":1790477685393,"version":"4.1.0"},"reference-count":50,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,12,26]],"date-time":"2019-12-26T00:00:00Z","timestamp":1577318400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      To assess the benefit of the recombinant human interleukin\u20101 receptor antagonist anakinra in treating pediatric patients with secondary hemophagocytic lymphohistiocytosis (\n                      <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                      )\/macrophage activation syndrome (\n                      <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>\n                      ) associated with rheumatic and nonrheumatic conditions.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A retrospective chart review of all anakinra\u2010treated patients with secondary\n                      <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                      \/\n                      <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>\n                      was performed at Children's of Alabama from January 2008 through December 2016. Demographic, clinical, laboratory, and genetic characteristics, outcomes data, and information on concurrent treatments were collected from the records and analyzed using appropriate univariate statistical approaches to assess changes following treatment and associations between patient variables and outcomes.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Forty\u2010four patients with secondary\n                      <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                      \/\n                      <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>\n                      being treated with anakinra were identified in the electronic medical records. The median duration of hospitalization was 15 days. The mean pretreatment serum ferritin level was 33,316 ng\/ml and dropped to 14,435 ng\/ml (57% decrease) within 15 days of the start of anakinra treatment. The overall mortality rate in the cohort was 27%. Earlier initiation of anakinra (within 5 days of hospitalization) was associated with reduced mortality (\n                      <jats:italic>P<\/jats:italic>\n                      = 0.046), whereas thrombocytopenia (platelet count &lt;100,000\/\u03bcl) and\n                      <jats:italic>\n                        <jats:styled-content style=\"fixed-case\">STXBP<\/jats:styled-content>\n                        2\n                      <\/jats:italic>\n                      mutations were both associated with increased mortality (\n                      <jats:italic>P<\/jats:italic>\n                      = 0.008 and\n                      <jats:italic>P<\/jats:italic>\n                      = 0.012, respectively). In considering patients according to their underlying diagnosis, those with systemic juvenile idiopathic arthritis (\n                      <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>\n                      ) had the lowest mortality rate, with no deaths among the 13 systemic\n                      <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>\n                      patients included in the study (\n                      <jats:italic>P<\/jats:italic>\n                      = 0.006). In contrast, those with an underlying hematologic malignancy had the highest mortality rate, at 100% (n = 3).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      These findings suggest that anakinra appears to be effective in treating pediatric patients with non\u2013malignancy\u2010associated secondary\n                      <jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>\n                      \/\n                      <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content>\n                      , especially when it is given early in the disease course and when administered to patients who have an underlying rheumatic disease.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.41103","type":"journal-article","created":{"date-parts":[[2019,9,12]],"date-time":"2019-09-12T09:14:49Z","timestamp":1568279689000},"page":"326-334","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":257,"title":["Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis"],"prefix":"10.1002","volume":"72","author":[{"given":"Esraa M.","family":"Eloseily","sequence":"first","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine and Assiut University Children's Hospital  Assiut Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Weiser","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine"}],"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 School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hilary","family":"Haines","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa L.","family":"Mannion","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew L.","family":"Stoll","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy","family":"Beukelman","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham School of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. 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