儿童系统性红斑狼疮合并巨噬细胞活化综合征1例报告并文献复习

A case of childhood systemic lupus erythematosus complicated with macrophage activation syndrome and literature review

目的探讨系统性红斑狼疮(SLE)合并巨噬细胞活化综合征(MAS)的临床特点及治疗。方法回顾分析1例SLE合并MAS患儿的诊治过程,并复习相关文献。结果患儿,女,8岁,确诊SLE 2年余,因发热1周入院。实验室检查发现两系血细胞减少,纤维蛋白原降低,三酰甘油增高,血清铁蛋白进行性增高;肝、脾进行性肿大;确诊SLE合并MAS。结论 SLE合并MAS病情进展迅速,危及患儿生命,早期不易诊断。

Objective To study the clinical features and treatments of childhood systemic lupus erythematosus（SLE） complicated with macrophage activation syndrome（MAS）. Methods Clinical data of a patient with SLE complicated with MAS were retrospectively analyzed, and relevant literatures were reviewed. Results An eight-year-old girl diagnosed as SLE two years ago was admitted to the hospital with complaint of high fever for over one week. Peripheral blood cytopenia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia and hepatolienomegaly occurred after admission gradually. Thus the child was diagnosed definitely as MAS. Conclusions SLE complicated with MAS is not rare. The disease progresses very rapidly and can be life-threatening, while early diagnosis is very difficult. The key to improve prognosis is early diagnosis and treatment.