Pathogenesis and long-term prognosis of Kawasaki disease : New therapeutic strategy using vasogenesis therapy

川崎病(KD)是一种原因不明的急性自限性全身性血管炎综合征，主要累及中小型动脉，尤其是冠状动脉。约3%-8%的儿童在静脉注射免疫球蛋白(IVIG)治疗后发生冠状动脉病变(CALS)，约4%的KD儿童发展为缺血性心脏病合并心肌梗死。根据临床特征和流行病学资料，KD可能是由一些易感宿主中非常常见的感染性因素引起的。我们利用Affymetrix基因芯片系统研究了KD患者和对照组之间基因表达的差异。一些遗传因素可能参与了KD的发病，一些血管生成因素可能参与了CAL的发生。近30年来2180例急性川崎病患者的心血管谱。在所有患者中，14.9%的患者被诊断为CALS。自1991年开始静脉注射免疫球蛋白治疗以来，CALS的发病率已降至8.7%。发生心肌梗死24例，死亡7例。对KD患者进行长期随访研究是必要的，并应注意早期动脉粥样硬化的可能发展。

Kawasaki disease (KD) is an acute, self limiting systemic vasculitis syndrome of unknown origin that mainly affects small and medium-sized arteries, particularly the coronary artery. From 3-8% of children develop coronary artery lesions (CALs) after intravenous immunoglobulin (IVIG) therapy, and approximately 4% of KD children develop ischemic heart disease with myocardial infarction. On the basis of clinical features and epidemiological data, KD might be caused by very common infectious agents in some susceptible hosts. We investigated the difference of gene expression between patients with KD and control subjects by use the Affymetrix GeneChip system. Some genetic factors might participate in pathogenesis of KD, and some angiogenesis factors might concern in incidence of CAL. The cardiovascular spectrum of 2180 patients with acute KD experienced over the last 30 years. Of all the patients, 14.9% were diagnosed as having CALs. From 1991 we started IVIG treatment, since then the incidence of CALs has declined to 8.7%. Myocardial infarction occurred in 24 patients, 7 of whom died. Long-term follow-up study is necessary in KD patients, and should pay attention to the possible development of premature atherosclerosis.