{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T20:54:19Z","timestamp":1775854459213,"version":"3.50.1"},"reference-count":34,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2020,9,3]],"date-time":"2020-09-03T00:00:00Z","timestamp":1599091200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>To evaluate the clinical and laboratory characteristics, prognostic factors, and outcome of adult rheumatic disease\u2010associated macrophage activation syndrome (MAS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A multicenter retrospective study was performed across 4 tertiary hospitals in China between January 1, 2017 to December 31, 2019.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 61 rheumatic disease patients with MAS enrolled into this retrospective clinical study. Fever and hyperferritinemia are the most frequent clinical feature and laboratory abnormality in MAS patients. Serum ferritin\u00a0&gt;\u00a06000\u00a0ng\/mL (odds ratio [OR]\u00a0=\u00a09.46, 95% CI\u00a0=\u00a02.53\u201047.13, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.005) and hemophagocytosis in bone marrow smear (OR\u00a0=\u00a011.12, 95%, CI\u00a0=\u00a03.29\u201050.65, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.001) were the 2 most prominent predictive factors indicating MAS occurrence. The 90\u2010day all\u2010cause mortality rate of all rheumatic disease patients with MAS was 22.9% (hazards ratio [HR]\u00a0=\u00a02.15, 95% CI\u00a0=\u00a00.81\u20106.78, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.05). Platelets\u00a0&lt;\u00a0100\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L (HR\u00a0=\u00a03.23, 95% CI\u00a0=\u00a02.51\u20104.81, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.01) and ferritin\u00a0&gt;\u00a06000ng\/mL (HR\u00a0=\u00a06.12, 95% CI\u00a0=\u00a02.93\u201016.27, <jats:italic>P<\/jats:italic>\u00a0=\u00a0.005) were independent predictors of poor outcome in rheumatic disease\u2010associated MAS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Macrophage activation syndrome could be a fatal complication in rheumatic disease. Patients presenting with unexplained fever, serum ferritin\u00a0&gt;\u00a06000\u00a0ng\/mL, hepatosplenomegaly and cytopenia at baseline should raise the suspicion of MAS. The presence of serum ferritin\u00a0&gt;\u00a06000\u00a0ng\/mL, hepatosplenomegaly and low number of platelets was associated with poor outcome.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1756-185x.13955","type":"journal-article","created":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T05:40:08Z","timestamp":1599198008000},"page":"1488-1496","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Clinical analysis of macrophage activation syndrome in adult rheumatic disease: A multicenter retrospective study"],"prefix":"10.1111","volume":"23","author":[{"given":"Yao","family":"Ke","sequence":"first","affiliation":[{"name":"Department of Rheumatology the First Affiliated Hospital of Nanjing Medical University  Nanjing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chengyin","family":"Lv","sequence":"additional","affiliation":[{"name":"Department of Rheumatology the First Affiliated Hospital of 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