A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis
A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis
复制标题
DOI：
10.1007/s11926-020-0882-1
复制
发表时间：
2020-02-05
影响因子：
5
通讯作者：
Arditi, Moshe
中科院分区：
文献类型：
--
作者：
Soni, Priya R.;Noval Rivas, Magali;Arditi, Moshe
综述目的：川崎（KD）是一种病因不明的儿童全身性血管炎，可导致冠状动脉瘤（CAA），如果不及时诊断，可导致长期心血管并发症和成人心脏病。高达20%的KD儿童对IVIG（主要治疗方法）无效，这突出表明需要新的治疗策略。在这里，我们回顾了该领域的最新发现，关于特定的病因，遗传协会和治疗策略的进展，以防止冠状动脉瘤。最近的发现最近发现使用干酪乳杆菌细胞壁提取物（LCWE）诱导的KD血管炎小鼠模型加速了KD病理生理学的研究，并有先进的治疗策略，包括IL-1 R拮抗剂Anakinra的临床试验。KD仍然是一种难以捉摸的儿科血管炎综合征，是美国和发达国家儿童获得性心脏病的主要原因。难治性KD联合治疗的进展以及对新遗传风险因素的进一步了解为该领域未来的研究工作奠定了坚实的基础。
Purpose of the Review Kawasaki disease (KD) is a childhood systemic vasculitis of unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel therapeutic strategies. Here we review the latest findings in the field regarding specific etiology, genetic associations, and advancements in treatment strategies to prevent coronary aneurysms. Recent Findings Recent discoveries using the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model have accelerated the study of KD pathophysiology and have advanced treatment strategies including clinical trials for IL-1R antagonist, Anakinra. KD remains an elusive pediatric vasculitis syndrome and is the leading cause of acquired heart disease among children in the USA and developed countries. Advancements in combination treatment for refractory KD with further understanding of novel genetic risk factors serve as a solid foundation for future research endeavors in the field.