川崎病发病机制及诊疗进展

Pathogenesis of Kawasaki Disease and Progress in Diagnosis and Treatment

摘要: 川崎病(Kawasaki disease, KD)又名皮肤黏膜淋巴结综合征，是一种病因不明的累及全身中小血管的系统性血管炎。以发热、皮疹、手足硬肿、黏膜改变等为主要表现，根据临床表现分为完全性川崎病(Complete Kawasaki disease, CKD)及不完全型川崎病(Incomplete Kawasaki disease, IKD)。该病好发于5岁以下儿童，尤其是6个月至2岁的幼儿，男女比例为1.8:1，是儿童时期最常见的获得性心脏病病因，最危险的并发症是冠状动脉损伤(Coronary artery lesion, CAL)，严重时可导致冠状动脉瘤(Coronary artery aneurysm, CAA)、心肌梗死，甚至猝死。因此，早发现、早诊断、早治疗对于KD尤为重要。本文针对川崎病最新发病机制研究、特殊类型的诊断及治疗方案进行总结，以期为KD临床诊疗提供依据。

Abstract: Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, which is a systemic vasculitis of unknown etiology affecting small and medium-sized blood vessels throughout the body. Its primary manifestations include fever, rash, hard swelling of the hands and feet, and mucosal changes. Based on clinical presentation, it is classified into complete Kawasaki disease (CKD) and incomplete Kawasaki disease (IKD). This disease predominantly affects children under 5 years old, particularly those aged 6 months to 2 years, with a male-to-female ratio of 1.8:1. It is the most common cause of acquired heart disease in childhood. The most dangerous complication is coronary artery lesion (CAL), which in severe cases can lead to coronary artery aneurysm (CAA), myocardial infarction, or even sudden death. Therefore, early detection, diagnosis, and treatment are crucial for KD. This article summarizes the latest research on the pathogenesis of Kawasaki disease, the diagnosis of special subtypes, and treatment strategies, aiming to provide a basis for the clinical diagnosis and treatment of KD.