Abstract: Objective　To study the clinical and laboratory features，treatment and outcome of rheumatic disease-associated macrophage activation syndrome（MAS） in children. Methods　We retrospectively analyzed the clinical and laboratory features，treatment and outcome of 75 patients with juvenile rheumatic disease-associated MAS. Results　Of the 75 MAS patients，32 with systemic juvenile idiopathic arthritis（SJIA），22 with systemic lupus erythematosus（SLE），15 withKawasaki disease（KD），5 with juvenile dermatomyositis（JDM）　and 1 with mixed connective tissue disease（MCTD） as primary diseases. The common clinical symptoms included fever（100%），hepatomegaly（73.3%），splenomegaly（49.3%），nervous system symptoms（45.3%），lymph node enlargement（42.7%） and hemorrhagic manifestations（24%）. There were decreases in white blood cell，platelet，hemoglobin and fibrinogen，and increases in serum ferritin，transaminase，lactate dehydrogenase and triglyceride. There was no significant difference between glucocorticoid pulse therapy and non-pulse therapy in the time of fever clearance or the proportion of death. There were 13 deaths in this group. The incidence of bleeding，nervous system involvement and respiratory failure was higher in the death group，and the death group had lower levels of fibrinogen and NK cells. Conclusion　If patients with rheumatic disease have persistent fever，hepatosplenomegaly，decreases in white blood cell，platelet and hemoglobin and increase in serum ferritin，the development of MAS should be considered. Bleeding，nervous involvement and severe hypofibrinogenemia suggest poor prognosis. Glucocorticoid therapy remains the first choice in the treatment of MAS，but the dose needs further study.

Key words: macrophage activation syndrome, systemic juvenile idiopathic arthritis, systemic lupus erythematosus, Kawasaki disease, child

摘要： 目的　总结儿童风湿病相关巨噬细胞活化综合征（MAS）临床和实验室特征、治疗及转归。方法　回顾性分析2008年1月至2019年11月重庆医科大学附属儿童医院75例MAS患儿的临床和实验室特征、治疗及转归。结果　MAS的基础疾病包括全身型幼年特发性关节炎（SJIA） 32 例、系统性红斑狼疮（SLE） 22例、川崎病（KD） 15 例、幼年皮肌炎（JDM） 5例和混合结缔组织疾病（MTCD） 1例。常见的临床症状有发热（100%）、肝大（73.3%）、脾肿大（49.3%）、神经系统症状（45.3%）、淋巴结肿大（42.7%）、出血（24.0%）。实验室检查显示三系下降，铁蛋白、转氨酶、乳酸脱氢酶、三酰甘油升高，纤维蛋白原降低。糖皮质激素冲击治疗和非冲击治疗在体温恢复正常时间及死亡比例方面差异无统计学意义。13例死亡，死亡组患儿出血、神经系统受累及呼吸衰竭发生率更高，纤维蛋白原及NK细胞更低。结论　风湿病患儿出现发热、肝脾肿大、血常规下降、铁蛋白升高需警惕MAS发生。出血、神经系统受累、严重低纤维蛋白原血症提示预后不良。糖皮质激素仍是治疗首选，剂量选择有待进一步研究。

关键词: 巨噬细胞活化综合征, 全身型幼年特发性关节炎, 系统性红斑狼疮, 川崎病, 儿童