{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T19:00:04Z","timestamp":1781290804516,"version":"3.54.1"},"reference-count":40,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ther Apher Dial"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Adult\u2010onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS) carries substantial mortality. The role of therapeutic plasma exchange (TPE) remains uncertain.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed patients with AOSD\u2010MAS treated with TPE at a single\u2010center.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>All five patients had arthralgia, a salmon\u2010pink rash, extreme hyperferritinemia, and multiorgan dysfunction including liver dysfunction. Prior to TPE, all patients received pulse glucocorticoids; three also received calcineurin inhibitors and one tocilizumab. TPE was initiated at a median of 12.0\u2009days after diagnosis; the patients underwent a median of six sessions without major adverse events. Following TPE, fever and rash resolved; C\u2010reactive protein (CRP), ferritin, and liver enzyme levels decreased without further corticosteroid escalation during and immediately after TPE. At 1\u2009month, four patients achieved CRP &lt;\u20091.0\u2009mg\/dL, four recovered with tapered immunosuppression. One patient died from cytomegalovirus infection despite MAS improvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Adjunctive TPE was associated with rapid clinical and biochemical improvement.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/1744-9987.70133","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T03:09:30Z","timestamp":1773025770000},"page":"389-396","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Adjunctive Therapeutic Plasma Exchange in Refractory Adult\u2010Onset Still's Disease Complicated by Secondary Macrophage Activation Syndrome: A Single\u2010Center Experience"],"prefix":"10.1002","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-7739-6648","authenticated-orcid":false,"given":"Masataka","family":"Ueda","sequence":"first","affiliation":[{"name":"Department of Nephrology Showa Medical University Graduate School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2303-5401","authenticated-orcid":false,"given":"Tomohiro","family":"Saito","sequence":"additional","affiliation":[{"name":"Department of Nephrology Showa Medical University Graduate School of Medicine  Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahide","family":"Mizobuchi","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Internal Medicine Showa Medical University Fujigaoka Hospital  Yokohama 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