Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis
Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis
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DOI：
10.1093/rheumatology/keq133
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发表时间：
2010-09-01
期刊：
影响因子：
5.5
通讯作者：
Yachie, Akihiro
中科院分区：
文献类型：
--
作者：
Shimizu, Masaki;Yokoyama, Tadafumi;Yachie, Akihiro
方法。对 5 名 MAS 并发 s-JIA 患者 (MAS/s-JIA)、10 名 EBV 感染所致 HLH (EBV-HLH) 患者、22 名 KD 患者和 28 名健康对照进行了分析。通过 ELISA 对血清中的细胞因子浓度（IL-18、IL-6、新蝶呤以及 I 型和 II 型 TNF-α 受体）进行定量。将结果与 MAS/s-JIA 的临床特征（包括铁蛋白浓度）进行比较。结果。 MAS/s-JIA患者血清IL-18浓度显着高于EBV-HLH或KD患者（P < 0.05）。 KD 患者血清 IL-6 浓度显着高于 EBV-HLH 或 MAS/s-JIA 患者。 EBV-HLH 患者的血清新蝶呤浓度显着高于 MAS/s-JIA 或 KD 患者。血清IL-18与以下疾病活动指标呈正相关：CRP、铁蛋白、乳酸脱氢酶和其他细胞因子（P < 0.05）。 s-JIA 患者的 IL-18 血清浓度在疾病非活动期仍然升高，而临床参数和其他细胞因子则正常化。结论。 IL-18 可能是 s-JIA 的重要介质。尽管血清 IL-18 浓度与疾病活动标志物相关，但即使疾病活动的其他标志物正常化，IL-18 浓度仍保持升高。血清 IL-18 浓度可能是疾病活动的一个有希望的指标。不同病因的 MAS/HLH 患者的细胞因子释放模式不同。监测细胞因子谱（包括 IL-18）可能有助于区分 MAS/HLH 和评估 s-JIA 疾病活动性。
Methods. Five patients with MAS complicating s-JIA (MAS/s-JIA), 10 with HLH due to EBV infection (EBV-HLH), 22 with KD and 28 healthy controls were analysed. Cytokine concentrations (IL-18, IL-6, neopterin and TNF-alpha receptor Types I and II) were quantified in serum by ELISA. Results were compared with clinical features of MAS/s-JIA, including ferritin concentrations.Results. Serum IL-18 concentrations in MAS/s-JIA patients were significantly higher than those in EBV-HLH or KD patients (P < 0.05). Serum IL-6 concentrations in KD patients were significantly higher than those in EBV-HLH or MAS/s-JIA patients. Serum neopterin concentrations in EBV-HLH patients were significantly higher than those in MAS/s-JIA or KD patients. Serum IL-18 correlated positively with the following measurements of disease activity: CRP, ferritin, lactate dehydrogenase and other cytokines (P < 0.05). Serum concentrations of IL-18 in s-JIA patients remained elevated in the inactive phase of disease, whereas clinical parameters and other cytokines normalized.Conclusions. IL-18 may be an important mediator in s-JIA. Although serum Il-18 concentrations correlated with markers of the disease activity, IL-18 concentrations remained elevated even when other markers of disease activity normalized. Serum IL-18 concentration may be a promising indicator of the disease activity. The cytokine release pattern in MAS/HLH is different among patients with different aetiologies. Monitoring the cytokine profile, including IL-18, may be useful for differentiation of MAS/HLH and evaluation of disease activity in s-JIA.