系统性红斑狼疮继发巨噬细胞活化综合征15例临床分析

The clinical characteristics of macrophage activation syndrome secondary to systemic lupus erythematosus

目的 通过分析总结系统性红斑狼疮（SLE）继发巨噬细胞活化综合征（MAS）的临床资料,提高对其的认识及诊治水平.方法 回顾性分析北京协和医院2011年7月至2014年12月收治的15例SLE继发MAS的临床资料,包括一般临床资料、实验室检查结果、治疗及预后.结果 15例SLE继发MAS患者中女性14例,男性1例,平均年龄28.07岁,发生MAS时SLE的平均病程为20.47个月,SLE疾病活动指数（SLEDAI）为18.4.15例患者MAS病程中均出现发热、血细胞减少、肝功能损伤,11例脾脏肿大,14例凝血功能障碍,8例出现神经精神症状.15例患者WBC、PLT减少,7例纤维蛋白原降低,11例铁蛋白升高,12例有骨髓吞噬血细胞现象.15例SLE患者继发MAS后均接受了大剂量糖皮质激素治疗,8例接受了糖皮质激素冲击治疗;13例使用了免疫抑制剂（包括环孢素A、他克莫司、环磷酰胺、吗替麦考酚酯）;10例应用了静脉免疫球蛋白;2例接受了依托泊苷治疗.15例SLE继发MAS患者经治疗后,14例MAS病情完全缓解,1例死亡.结论 SLE继发MAS多发于病程较短、SLE病情高度活动的青年女性,临床上发热、脾脏肿大、血细胞减少、凝血功能障碍、肝功能损伤突出,早期诊断和早期、精准治疗是改善预后的关键.

Objective To investigate the clinical features of macrophage activation syndrome （MAS） associated with systemic lupus erythematosus （SLE）.Method The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July 2011 to December 2014 were retrospectively analyzed.Results Fourteen patients were female.The average age was 28.07.When MAS occurred,the average duration of SLE was 20.47 months,and the average SLE disease activity index （SLEDAI） was 18.4.All 15 patients developed fever,hematocytopenia and impaired liver function in the course of MAS,while patients with splenomegaly,coagulation disorders and neuropsychiatric symptoms were 11,14 and 8,respectively.All 15 patients presented leukocpenia and thrombocytopenia.Hypofibrinogenemia,elevated ferritin and hemophagocytosis in bone marrow were respectively observed in 7,11 and 12 patients.Glucocorticoids were used in all patients,among whom eight received pulse methylprednisolone therapy.Thirteen patients were treated with immunosuppressants,including cyclosporine A,tacrolimus,cyclophosphamide and mycophemolate mofetil.Complete remission was achieved in 14 patients.One patient died of MAS.Conclusions In patients with SLE,MAS was most commonly seen in young females with short SLE duration and active disease.Fever,splenomegaly,hematocytopenia,coagulation disorders and liver damage are the most remarkable clinical manifestations.Early diagnosis and intensive therapy are the key parts to improve clinical outcome.