巨噬细胞活化综合征11例临床分析

Eleven patients with macrophage activation syndrome： case reports

目的 通过分析11例巨噬细胞活化综合征（MAS）的临床及实验室特点,提高对该病的认识.方法 分析北京大学人民医院风湿免疫科近6年收治的11例MAS患者的临床资料,主要包括基础疾病、MAS发生诱因、临床表现、实验室特征、治疗方案及预后等,并结合文献进行分析讨论.结果 11例患者除1例为男性外均为女性患者,发生MAS时的年龄为19 ～ 65岁,平均（37±17）岁.11例患者包括SLE和AOSD各4例,另有3例为SS.50％以上患者MAS与自身免疫病同时诊断.临床以高热（100％,11/11）、淋巴结肿大（45％,5/11）及脾大（45％,5/11）为主要表现.实验室检查方面,以血细胞减少（91％,10/11）和铁蛋白升高（100％,11/11）为主,73％（8/11）患者铁蛋白＞2 000μg/L.高TG血症达一半以上（6/11）.纤维蛋白原减低占50％（5/10）,其中2例发生弥漫性血管内凝血.4例中有3例患者NK细胞活性减低或缺失,3例可溶性CD25抗原（sCD25）全部升高（3/3）.82％（9/11）的患者骨髓检查见噬血细胞现象.其他系统损害以肝功能异常（64％,7/11）和肺部感染（36％,4/11）多见.11例患者均应用糖皮质激素治疗,其中8例应用激素冲击治疗,7例联合应用免疫抑制剂.82％（9/11）的患者经治疗好转,2例死亡.随访生存患者病情稳定,无MAS复发.结论 免疫病初次发病即出现MAS并非少见.高热、铁蛋白＞2 000 μg/L及sCD25升高在MAS中常见.足量激素或激素冲击联合免疫抑制剂治疗可改善MAS预后.

Objective To analyze the clinical features and laboratory data of 11 patients with macrophage activation syndrome （MAS）.Methods Data of 11 patients with MAS from last 6 years in the Department of Rheumatology,Peking University People＆#39;s Hospital were collected.The underlying diseases,clinical features,laboratory findings,treatment outcomes and prognosis were analyzed.Results Eleven patients were mostly female （aged from 19 to 65 years,37±17）.The underlying diseases were systemic lupus erythematosus （n=4）,adult-onset Still＆#39;s disease （n=4） and Sj（o）gren＆#39;s Syndrome （n=3）.MAS was the first symptom in nearly half patients.MAS was characterized by high fever （100%,11/11）,lymphadenopathy （45%,5/11） and hypersplenomegaly （45%,5/11）.Laboratory data showed cytopenia （91%,10/11）,serum ferritin elevation （100%,11/11）,hypofibrinogenemia （50%,5/10）,hypertriglyceridemia （55%,6/11） and hemophagocytosis in bone marrow （82%,9/11）.Low NK-cell activity （3/4） and elevation of sCD25 （3/3） was the specific manifestation in MAS.Pulmonary infection （36%,4/11） and hepatic malfunction （64%,7/11） were common.All patients received corticosteroid,and pulses corticosteroid therapy （methylprednisolone 200-500 mg/d） was used in 8 patients.Seven were treated with combined immunosuppressants.Eighty-two percent recovered,two patients died.Alive patients were stable during the follow-up.Conclusion MAS is commonly seen in the initial onset of autoimmune disease.MAS occurrs simultaneously with multiple systemic diseases and is often difficult to distinguish from active autoimmune diseases.High fever,remarkable serum ferritin elevation and elevation of sCD25 are more common in MAS secondary to autoimmune disease.Corticosteroids,immunosuppressant and intravenous immunoglobulin may improve the prognosis of MAS.