Management of Kawasaki disease.
Management of Kawasaki disease.
复制标题
川崎病的治疗。
DOI：
10.1136/archdischild-2012-302841
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发表时间：
2014-01
影响因子：
5.2
通讯作者：
Brogan PA
中科院分区：
文献类型：
--
作者：
Eleftheriou D;Levin M;Shingadia D;Tulloh R;Klein NJ;Brogan PA
川崎（KD）是一种急性自限性炎症性疾病，与血管炎相关，主要累及中型动脉，特别是冠状动脉。在发达国家，KD是儿童获得性心脏病的最常见原因。KD的病因仍然未知，目前认为一种或多种尚未鉴定的感染原在遗传易感个体中诱导强烈的炎症宿主反应。遗传学研究已经在不同种族人群中确定了KD及其后遗症的几种易感基因，包括FCGR 2A、CD 40、ITPKC、FAM 167 A-BLK和CASP 3，以及影响静脉内免疫球蛋白（IVIG）反应和动脉瘤形成的基因，如FCGR 3B和转化生长因子（TGF）β途径基因。IVIG和阿司匹林在治疗上是有效的，但最近的临床试验和荟萃分析表明，在IVIG抵抗风险最高的严重病例中，在IVIG中加入皮质类固醇有利于预防冠状动脉瘤（CAA）。然而，在日本以外，预测IVIG抵抗的临床评分表现不佳。此外，证据基础没有提供明确的指导，皮质类固醇方案是最有效的。其他治疗（包括抗TNF α）也可能对IVIG耐药KD起作用。不管这些警告，很明显，减少急性KD炎症的治疗可以改善结果。本文总结了KD发病机制和治疗的理解的最新进展，并提供了一种方法来管理KD患者在英国根据这些进展。
Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.
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影响因子：
3.6
作者：
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通讯作者：
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