Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2.
Disruption of vascular endothelial homeostasis in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The dynamic roles of angiopoietin-1 and -2.
复制标题
系统性幼年特发性关节炎相关巨噬细胞活化综合征中血管内皮稳态的破坏：血管生成素-1和-2的动态作用。
DOI：
10.1016/j.cyto.2016.02.008
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发表时间：
2016
期刊：
影响因子：
3.8
通讯作者：
Yachie A.
中科院分区：
文献类型：
--
作者：
Tasaki Y;Shimizu M;Inoue N;Mizuta M;Nakagishi Y;Wada T;Yachie A.
为了评估血管生成素(Ang)-1和Ang-2在系统性幼年特发性关节炎（s-JIA）相关巨噬细胞激活综合征（MAS）中的作用并探讨其血清水平的临床意义，我们检测了51例s-JIA患者、11例多关节性关节炎（poly-JIA）患者、12例病毒相关噬血细胞综合征（VAHS）患者、12例川崎病（KD）患者和15例年龄匹配的健康对照（HC）患者的血清水平。结果与MAS的临床特征进行比较。与活动期和非活动期相比，MAS期血清ang1水平明显降低。与活动期和非活动期相比，MAS期血清Ang-2/1比值显著升高。MAS发病时Ang-2/1比值迅速升高。血清Ang-1和Ang-2/1比值与疾病活动性指标（包括AST和LDH）显著相关。在VAHS患者中也观察到Ang-2/1失调，而在大多数KD病例中未观察到。在MAS中，血管内皮功能的稳态被ang1和ang2破坏。血清Ang-1水平和Ang-2/1比值可能是MAS疾病活动性的有希望的指标。
To assess the role of angiopoietin (Ang)-1 and Ang-2 and to investigate the clinical significance of serum levels of them in systemic juvenile idiopathic arthritis (s-JIA)-associated macrophage activation syndrome (MAS), we determined these levels in 51 patients with s-JIA, 11 patients with polyarticular JIA (poly-JIA), 12 patients with virus associated hemophagocytic syndrome (VAHS), 12 patients with Kawasaki disease (KD), and 15 age-matched healthy controls (HC). The results were compared with clinical features of MAS. During the MAS phase, serum Ang-1 levels were significantly decreased compared with those during the active and inactive phases. Serum Ang-2/1 ratio were significantly elevated during the MAS phase, compared with those during the active and inactive phases. There was a rapid increase in the Ang-2/1 ratio at the onset of MAS. Serum Ang-1 and the Ang-2/1 ratio significantly correlated with measures of disease activity, including AST and LDH. Ang-2/1 dysregulation was also observed in patients with VAHS, whereas not observed in most cases of KD. The homeostasis of vascular endothelial function by Ang-1 and Ang-2 is disrupted in MAS. Serum Ang-1 levels and the Ang-2/1 ratio might represent promising indicators of disease activity for MAS.