Division of Rheumatology, Department of Medicine, Showa University School of Medicine, 1-5-8 Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan.
Clin Rheumatol. 2012 May;31(5):853-60. doi: 10.1007/s10067-012-1952-1. Epub 2012 Feb 10.
To investigate the characteristics of patients with adult-onset Still's disease (AOSD), serum cytokines and chemokines were measured to examine their associations with systemic manifestations of AOSD, especially hemophagocytic syndrome (HPS). Nineteen patients diagnosed with AOSD were enrolled. Serial serum samples were obtained from patients with AOSD in both active and inactive stages and controls. The concentrations of cytokines and chemokines, including IL-18, soluble IL-2 receptor (sIL-2R), CX3CL1, CXCL8, CXCL10, CCL2, and CCL3, were determined using enzyme-linked immunosorbent assay. Multivariate analysis was used to evaluate the correlations among serum chemokine levels, disease activity, and the clinical features of AOSD. Significantly higher serum levels of all cytokines and chemokines were observed in patients with active, untreated AOSD than in controls. The level of CX3CL1, but not other chemokines, was elevated in AOSD patients and was positively correlated with clinical activity and the levels of CRP, ferritin, IL-18, and sIL-2R. Among the 19 patients with AOSD, four patients also had HPS. Serum CX3CL1 and ferritin were significantly higher in AOSD patients with HPS than in those without HPS. The serum CX3CL1 level may be used as a clinical marker to assess the disease activity of AOSD, and high serum CX3CL1 and ferritin in patients with AOSD reflected the presence of HPS. The association between the chemokine profile and distinct clinical manifestations or various patterns of disease progression indicates that the pathogenesis of AOSD is heterogeneous.
为了探究成人Still 病（AOSD）患者的特征，我们测量了血清细胞因子和趋化因子的水平，以研究其与 AOSD 的全身表现，尤其是噬血细胞综合征（HPS）的相关性。共纳入 19 例 AOSD 患者。采集 AOSD 患者活动期和缓解期及健康对照者的连续血清样本。采用酶联免疫吸附试验检测细胞因子和趋化因子（包括 IL-18、可溶性白细胞介素 2 受体[sIL-2R]、CX3CL1、CXCL8、CXCL10、CCL2 和 CCL3）的浓度。采用多元分析评估血清趋化因子水平与疾病活动度及 AOSD 临床特征之间的相关性。与健康对照组相比，活动期未经治疗的 AOSD 患者的所有细胞因子和趋化因子血清水平均显著升高。CX3CL1 水平升高（但其他趋化因子无此变化），且与临床活动度及 CRP、铁蛋白、IL-18 和 sIL-2R 水平呈正相关。19 例 AOSD 患者中，4 例还合并有 HPS。与无 HPS 的 AOSD 患者相比，有 HPS 的 AOSD 患者的血清 CX3CL1 和铁蛋白水平显著升高。血清 CX3CL1 水平可作为评估 AOSD 疾病活动度的临床标志物，且 AOSD 患者血清 CX3CL1 和铁蛋白水平升高提示存在 HPS。趋化因子谱与不同临床表现或疾病进展的不同模式之间的相关性表明，AOSD 的发病机制存在异质性。
