环孢素、大剂量甲泼尼龙、丙种球蛋白联合治疗儿童巨噬细胞活化综合征并肝衰竭2例及文献复习

Ciclosporin,High Dose Methylprednisolon and Gammaglobulin in Children with Macrophage Activation Syndrom and Acute Liver Failure:A Follow-up Analysis of Two Cases and Literature Review

目的:总结儿童巨噬细胞活化综合征并肝衰竭的临床特点,为临床提供诊疗经验。方法:对青岛市儿童医院风湿免疫科2011年至2012年诊治的2例巨噬细胞活化综合征并肝衰竭患儿的临床资料进行分析、总结,并复习相关文献。结果:2例患儿均继发于全身型幼年特发性关节炎,肝衰竭为突出临床表现,经环孢素、大剂量甲泼尼龙、丙种球蛋白联合治疗后,病情缓解,随访超过6个月无复发。结论:巨噬细胞活化综合征并肝衰竭患儿病情凶险,病死率高,积极治疗可改善预后。环孢素、大剂量甲泼尼龙、丙种球蛋白联合治疗可作为一线治疗方案。

Objective： To provide experience of diagnosis and treatment for clinicians through summarizing the clinical characteristics of macrophage activation syndrome and acute liver failure in children. Methods： Clinical data of two cases in department of rheumatology of Qingdao Children＇s Hospital from 2011 to 2012 were analyzed and summarized. Related literatures were also studied. Results： The disease of both cases was secondary to systemic onset juvenile idiopathic arthritis, and acute liver failure was their key clinical manifestation. Two cases were improved with ciclosporin combined with high dose methylprednisolone and gammaglobulin, and were followed up for more than six months without recurrence. Conclusions： Acute liver failure in children with macrophage activation syndrome is dangerous and has high mortality. Ciclosporin combined with high dose methylprednisolone and gammaglobulin may be the first-line therapy.