{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T16:04:39Z","timestamp":1777392279041,"version":"3.51.4"},"reference-count":25,"publisher":"The Journal of Rheumatology","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2010,5]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>To elucidate the cytokine profiles of macrophage activation syndrome (MAS) in relation to underlying rheumatic diseases and prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>The clinical features and laboratory data of 18 patients with MAS and rheumatic diseases were retrospectively analyzed. Serum levels of macrophage colony-stimulating factor (M-CSF), interleukin 18 (IL-18), tumor necrosis factor-\u03b1, interleukin 6, interferon-\u03b3, ferritin, and \u00df<jats:sub>2<\/jats:sub>-microglobulin (\u00df<jats:sub>2<\/jats:sub>m) were measured. These data were compared between underlying diseases and between those who died and those who recovered.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>Of the 18 patients with MAS, 9 had underlying systemic lupus erythematosus (SLE), 7 had adult-onset Still\u2019s disease (AOSD), 1 had rheumatoid arthritis (RA), and 1 had antiphospholipid syndrome. Three patients with SLE and 1 patient with RA died. The serum M-CSF and IL-18 levels were substantially elevated in all the patients. In the patients with SLE, the M-CSF level was higher than the IL-18 level (median: 4879 vs 1341 pg\/ml, p = 0.0054), and it was the reverse in the patients with AOSD (5883 vs 228,350 pg\/ml, p = 0.0017). The serum M-CSF and \u00df<jats:sub>2<\/jats:sub>m levels were significantly higher in the patients who died than in those who recovered (M-CSF: 18,245 vs 3404 pg\/ml, p = 0.019; \u00df<jats:sub>2<\/jats:sub>m: 18.8 vs 5.4 mg\/dl, p = 0.0058).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>The cytokine profiles associated with MAS differed between patients with SLE and patients with AOSD. The patients with SLE showed a prominent increase in serum M-CSF levels, as did the patients with AOSD in serum IL-18 level. Patients who died had higher serum M-CSF and \u00df<jats:sub>2<\/jats:sub>m levels, and this suggests that aggressive treatment for patients with MAS and these profiles should be promptly started.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.090662","type":"journal-article","created":{"date-parts":[[2010,3,15]],"date-time":"2010-03-15T22:56:18Z","timestamp":1268693778000},"page":"967-973","source":"Crossref","is-referenced-by-count":79,"title":["Cytokine Profiles of Macrophage Activation Syndrome Associated with Rheumatic 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