Early progression of atherosclerosis in children with chronic infantile neurological cutaneous and articular syndrome
Early progression of atherosclerosis in children with chronic infantile neurological cutaneous and articular syndrome
复制标题
慢性婴儿神经皮肤关节综合征儿童动脉粥样硬化的早期进展
DOI：
10.1093/rheumatology/keu180
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发表时间：
2014
期刊：
影响因子：
5.5
通讯作者：
Hara T
中科院分区：
文献类型：
--
作者：
Yamamura K;Takada H;Uike K;Nakashima Y;Hirata Y;Nagata H;Takimoto T;Ishimura M;Morihana E;Ohga S;Hara T
目的：慢性炎症在动脉粥样硬化的发展中起关键作用。在RA患者中已经报道了动脉粥样硬化的早期进展。Cryopyrin相关周期性综合征（CAPS）是由NLRP 3基因突变引起的常染色体显性遗传的自身炎症性疾病。慢性婴儿神经性皮肤和关节（CINCA）综合征是CAPS最严重的形式，患者表现出皮疹、发热、葡萄膜炎和关节表现的早期发作。然而，目前还没有关于CAPS患者动脉粥样硬化的报道。本研究的目的是评估CINCA综合征患者动脉粥样硬化的发展。方法：颈动脉内膜中层厚度（IMT），硬度参数β，对3例CINCA综合征患者（平均年龄9.0岁）进行踝臂指数（ABI）和压力波传导速度（PWV）的超声检查结果：CINCA综合征患者颈动脉IMT、僵硬度参数β和PWV均显著高于健康对照组[0.51 mm（www.example.com）vs0.44 mm（P <0.05）]，而对照组颈动脉IMT、僵硬度参数β和PWV均显著高于健康对照组[0.05 mm（www.example.com）vs0.05 mm（P <0.05）]s.d.0.05意义（0.04），P= 0.0021; 6.1（s.d.1.7）vs3.9（1.0），P= 0.0018; 1203 cm/s（s.d.328）vs855（114），P= 0.017]。这项研究的结果强调了慢性炎症在动脉粥样硬化发展中的重要性。对青年CINCA综合征患者动脉粥样硬化的进一步分析，可能为进一步了解心血管疾病的发病机制提供依据。
Objective.Chronic inflammation plays a key role in the development of atherosclerosis. Early progression of atherosclerosis has been reported in patients with RA. Cryopyrin-associated periodic syndromes (CAPS) are autosomal dominant autoinflammatory disorders caused by heterozygousNLRP3gene mutations. Chronic infantile neurological cutaneous and articular (CINCA) syndrome is the most severe form of CAPS and patients display early onset of rash, fever, uveitis and joint manifestations. However, there has been no previous report on atherosclerosis in patients with CAPS. The objective of this study is to assess the development of atherosclerosis in patients with CINCA syndrome.Methods.Intima–media thickness (IMT) of the carotid arteries, stiffness parameter β, ankle brachial index (ABI) and pressure wave velocity (PWV) were evaluated by ultrasonography in 3 patients with CINCA syndrome [mean age 9.0 years (s.d.5.3)] and 19 age-matched healthy controls [9.3 years (s.d.4.3)].Results.The levels of carotid IMT, stiffness parameter β and PWV in CINCA syndrome patients were significantly higher than those in healthy controls [0.51 mm (s.d.0.05)vs0.44 (0.04),P= 0.0021; 6.1 (s.d.1.7)vs3.9 (1.0),P= 0.0018; 1203 cm/s (s.d.328)vs855 (114),P= 0.017, respectively].Conclusion.Patients with CINCA syndrome showed signs of atherosclerosis from their early childhood. The results of this study emphasize the importance of chronic inflammation in the development of atherosclerosis. Further analysis on atherosclerosis in young patients with CINCA syndrome may provide more insights into the pathogenesis of cardiovascular disease.