儿童川崎病相关巨噬细胞活化综合征的临床诊治

Clinical diagnosis and treatment of Kawasaki disease-associated macrophage activation syndrome in children

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。

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.