Department of Pediatrics and Department of Perinatal and Pediatric Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Rheumatology (Oxford). 2014 Oct;53(10):1783-7. doi: 10.1093/rheumatology/keu180. Epub 2014 May 11.
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 heterozygous NLRP3 gene 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.
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)].
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) vs 0.44 (0.04), P = 0.0021; 6.1 (S.D. 1.7) vs 3.9 (1.0), P = 0.0018; 1203 cm/s (S.D. 328) vs 855 (114), P = 0.017, respectively].
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.
慢性炎症在动脉粥样硬化的发展中起着关键作用。已有报道称，类风湿关节炎（RA）患者存在动脉粥样硬化的早期进展。 冷吡啉相关周期性综合征（CAPS）是一种常染色体显性自身炎症性疾病，由 NLRP3 基因突变引起。慢性婴儿神经皮肤关节综合征（CINCA）是 CAPS 中最严重的形式，患者表现为皮疹、发热、葡萄膜炎和关节表现的早期发病。然而，此前尚无 CAPS 患者发生动脉粥样硬化的报道。本研究旨在评估 CINCA 综合征患者的动脉粥样硬化发展情况。
通过超声评估 3 例 CINCA 综合征患者（平均年龄 9.0 岁，标准差 5.3）和 19 名年龄匹配的健康对照者（9.3 岁，标准差 4.3）的颈动脉内-中膜厚度（IMT）、僵硬参数β、踝臂指数（ABI）和脉搏波速度（PWV）。
CINCA 综合征患者的颈动脉 IMT、僵硬参数β和 PWV 水平明显高于健康对照组[0.51mm（标准差 0.05）比 0.44（0.04），P=0.0021；6.1（标准差 1.7）比 3.9（1.0），P=0.0018；1203cm/s（标准差 328）比 855（114），P=0.017]。
CINCA 综合征患者从儿童早期就出现了动脉粥样硬化迹象。本研究结果强调了慢性炎症在动脉粥样硬化发展中的重要性。对 CINCA 综合征年轻患者的动脉粥样硬化进一步分析可能为心血管疾病的发病机制提供更多见解。
