儿童川崎病相关巨噬细胞活化综合征的临床诊治

doi:10.12372/jcp.2022.23e0317

摘要/Abstract

摘要：

川崎病是一种系统性血管炎，巨噬细胞活化综合征是儿童风湿性免疫疾病的危重并发症，其发病基础为细胞因子风暴，进展快、病死率高，川崎病相关巨噬细胞活化综合征早期识别困难，主要特点为持续发热、脾肿大、铁蛋白水平升高和血小板减少等。目前没有统一诊断标准，主要参照全身型幼年特发性关节炎合并巨噬细胞活化综合征和原发性噬血细胞性淋巴组织细胞增多症的诊断标准，同时应与川崎病休克综合征和儿童多系统炎症综合征相鉴别。治疗以糖皮质激素为主要药物，反应不佳者可以使用环孢素等传统免疫抑制剂，生物制剂及靶向药物治疗具有前景。

关键词: 川崎病, 巨噬细胞活化综合征, 细胞因子风暴, 儿童

Abstract:

Kawasaki disease is a systemic vasculitis. Macrophage activation syndrome is a severe complication of pediatric rheumatic immune diseases, which is characterized by cytokine storms and has a rapid progression and high mortality rate. Early identification of Kawasaki disease-associated macrophage activation syndrome is difficult, and its main features include persistent fever, splenomegaly, elevated ferritin levels, and decreased platelets. At present, there is no uniform diagnostic criteria for this disease, which mainly refers to the diagnostic criteria of systemic juvenile idiopathic arthritis with macrophage activation syndrome and primary hemophagocytic lymphohistiocytosis. Meanwhile, this disease should be distinguished from Kawasaki disease shock syndrome and childhood multisystem inflammatory syndrome. Glucocorticoids are the main treatment drugs, and patients with poor response can use traditional immunosuppressants such as cyclosporine. Biologic agents and targeted drugs have broad prospects.

Key words: Kawasaki disease, macrophage activation syndrome, cytokine storm, child

郝胜, 黄敏. 儿童川崎病相关巨噬细胞活化综合征的临床诊治[J]. 临床儿科杂志, 2023, 41(7): 486-491.

HAO Sheng, HUANG Min. Clinical diagnosis and treatment of Kawasaki disease-associated macrophage activation syndrome in children[J]. Journal of Clinical Pediatrics, 2023, 41(7): 486-491.

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项目	KD-MAS	KDSS	MIS-C	SJIA-MAS
诱因/原发病	KD	KD	COVID-19	SJIA
发生率	2%以下	6%	<0.01%	约10%
脾肿大	常见	无	无	常见
铁蛋白	明显升高	正常	升高	明显升高
淋巴细胞	正常	正常	降低	正常
血小板	由高到低	正常或升高	正常或升高	正常或降低
肝酶	明显升高	正常或升高	正常或升高	明显升高
血沉	由高到低	升高	正常	由高到低
骨髓吞噬现象	常见	少见	少见	常见
累及系统	多系统（肝、血液、神经等）	循环系统	多系统（肺、心肌、胃肠道等）	多系统（肝、血液、神经等）