幼年特发性关节炎全身型合并巨噬细胞活化综合征4例报告并文献复习

Four cases report of macrophage activation syndrome with systemic onset juvenile idiopathic arthritis in children

目的总结巨噬细胞活化综合征(macrophage activation syndrome,MAS)的临床特征及误诊原因,以提高对该病的认识。方法回顾性分析54例幼年特发性关节炎全身型(systemic onset juvenile idiopathic arthritis,SO-JIA)合并MAS患儿的临床症状、体征、辅助检查及病情进展、诊断、治疗及预后。结果54例SOJIA患儿中4例并发MAS(7.4%)。临床特征有:持续高热、肝脾淋巴结增大、肝功能急剧恶化、皮肤黏膜易出血、外周血三系减少、中枢神经系统功能障碍、血沉进行性下降。结论MAS是SOJIA的一个致死性并发症,起病突然,进展迅速,病死率高。在临床工作中需提高对其的认识,避免误诊。

Objectives To summarize the clinical features of macrophage activation syndrome （MAS） and causes of misdiagnosis for further understanding this disease. Methods The clinical and laboratory features, diagnosis, treatment and outcome records from patients in last three years in our institute were retrospectively reviewed. Results Four patients （1 boy and 3 girls） were confirmed MAS with systemic onset juvenile idiopathic arthritis （SOJIA）（7.4% ）. The common clinical features were high fever, hepatosplenomegaly, lymphadenopathy, hepatic dysfunction, mucosal hemor rhage, pancytopenias, neurological involvement, erythrocyte sedimentation rate （ESR） progressely decreased. Conclusions MAS was a lethal complication of SOJIA, which characterized with sudden onset, rapid progress, and high mortality. Early diagnosis and treatment will benefit to prognosis.