Division of Pediatric Infectious Diseases and Immunology; Burns and Allen Research Institute, Cedars-Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, CA, 90048, USA.
Department of Biomedical Sciences-Infectious and Immunologic Diseases Research Center (IIDRC), Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA.
Curr Rheumatol Rep. 2020 Feb 5;22(2):6. doi: 10.1007/s11926-020-0882-1.
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 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.
川崎病（KD）是一种病因不明的儿童全身血管炎，可导致冠状动脉瘤（CAA），如果诊断不及时，可导致长期心血管并发症和成人心脏疾病。高达 20%的 KD 患儿对 IVIG（主要治疗方法）无反应，这凸显了需要新的治疗策略。本文综述了该领域在特定病因、遗传关联和治疗策略进展方面的最新发现，以预防冠状动脉瘤。
使用干酪乳杆菌细胞壁提取物（LCWE）诱导的川崎病血管炎小鼠模型的最新发现，加速了川崎病病理生理学的研究，并推进了治疗策略，包括白细胞介素-1R 拮抗剂阿那白滞素的临床试验。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。对于难治性 KD 的联合治疗的进展，以及对新的遗传风险因素的进一步了解，为该领域的未来研究奠定了坚实的基础。
