Cytokine Profiles of Macrophage Activation Syndrome Associated with Rheumatic Diseases
Cytokine Profiles of Macrophage Activation Syndrome Associated with Rheumatic Diseases
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DOI：
10.3899/jrheum.090662
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发表时间：
2010-05-01
影响因子：
3.9
通讯作者：
Inokuma, Shigeko
中科院分区：
文献类型：
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作者：
Maruyama, Junko;Inokuma, Shigeko
目标。目的：探讨巨噬细胞激活综合征（MAS）与风湿性疾病及预后的关系。回顾性分析18例MAS合并风湿病患者的临床特点及实验室资料。测定血清巨噬细胞集落刺激因子（m - csf）、白细胞介素18 （IL-18）、肿瘤坏死因子-a、白细胞介素6、干扰素γ、铁蛋白和β(2)-微球蛋白（β (2)m）的水平。这些数据在潜在疾病之间以及死亡和康复之间进行了比较。在18例MAS患者中，9例患有潜在系统性红斑狼疮（SLE）， 7例患有成人发病的斯蒂尔氏病（AOSD）， 1例患有类风湿性关节炎（RA）， 1例患有抗磷脂综合征。3例SLE患者和1例RA患者死亡。所有患者血清M-CSF和IL-18水平均显著升高。SLE患者M-CSF水平高于IL-18水平（中位数：4879 vs 1341 pg/ml， p = 0.0054）， AOSD患者则相反（中位数：5883 vs 228,350 pg/ml, p = 0.0017）。死亡患者血清m - csf和β (2)m水平明显高于康复患者（m - csf: 18245 vs 3404 pg/ml, p = 0.019; β (2)m: 18.8 vs 5.4 mg/dl, p = 0.0058）。SLE患者和AOSD患者与MAS相关的细胞因子谱不同。SLE患者血清M-CSF水平显著升高，AOSD患者血清IL-18水平显著升高。死亡患者的血清m - csf和β (2)m水平较高，这表明应立即开始对MAS患者和这些特征进行积极治疗。（2010年3月15日首次发布；J Rheumatol 2010:37:967-73; doi:10.3899/ jrheumat. 090662）
Objective. To elucidate the cytokine profiles of macrophage activation syndrome (MAS) in relation to underlying rheumatic diseases and prognosis.Methods. 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-a, interleukin 6, interferon-gamma, ferritin, and beta(2)-microglobulin (beta(2)m) were measured. These data were compared between underlying diseases and between those who died and those who recovered.Results. Of the 18 patients with MAS, 9 had underlying systemic lupus erythematosus (SLE), 7 had adult-onset Still's 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 beta(2)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; beta(2)m: 18.8 vs 5.4 mg/dl, p = 0.0058).Conclusion. 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 beta(2)m levels, and this suggests that aggressive treatment for patients with MAS and these profiles should be promptly started. (First Release March 15 2010; J Rheumatol 2010:37:967-73; doi:10.3899/jrheum.090662)