成人斯蒂尔病与巨噬细胞活化综合征关联性的临床分析

Clinical analysis of the relevance between adult-onset Still＇s disease and macrophage activation syndrome

目的探讨成人斯蒂尔病（AOSD）与巨噬细胞活化综合征（MAS）的关系。方法选择AOSD组为78例资料完整的AOSD；MAS组是从26例有组织学证据的噬血细胞综合征的随访治疗中确定11例为风湿免疫疾病相关的噬血细胞综合征。对以上患者的临床表现和实验室资料进行分析。结果在AOSD组78例中，有9例（占12％）在使用治疗之前可以诊断为MAS，但无噬血组织学依据。在11例有噬血现象的MAS中，AOSD6例，脂膜炎2例，系统性红斑狼疮、皮肌炎、系统性血管炎各1例。脾脏肿大、白细胞减低、贫血、血小板下降、高甘油三酯是AOSD出现MAS的相关临床指标。结论AOSD继发MAS的现象比较常见，严重者可以有组织学的噬血表现。AOSD出现脾脏增大、血细胞降低时，需要作MAS的相关检查，包括骨髓检查以及甘油三酯、纤维蛋白原、自然杀伤（NK）细胞活性等，以便及时诊断MAS。

Objective To explore the relationship between Adult-onset Still＇s disease （AOSD） and macrophage activation syndrome （MAS）. Methods A total of 78 patients with AOSD who had completed medical information were included in this study. Eleven patients who were diagnosed as rheumatic disease associated hemophagocytic syndrome among 26 patients who had hemophagocytic syndrome with histological evidence consisted of the MAS group. Clinical and laboratory data were analyzed in 78 patients with AOSD and 11 patients with MAS. Results Among 78 cases of AOSD, 9 patients （12%） could be diagnosed as MAS but didn＇t have hemophagocytic histological evidence. In the 11 MAS cases with hemophagocytic phenomenon, 6 patients fulfilled the diagnostic criteria of AOSD, 2 cases with panniculitis, 1 case with SLE, 1 case of dermatomyositis and 1 case of systemic vasculitis. Logistic analysis showed that splenomegaly （OR=2.13, 95%CI=1.11-3.42）, leukopenia （0R=3.57, 95%CI=2.30-4.86）, anaemia （0R=0.85, 95%CI=1.03-2.76）, thrombocytopenia （ OR =2.98, 95% CI= 1.17-4.30 ） and hypertriglyeeridemia （ OR = 1.66, 95% CI= 1.02-2.74 ） were associated with development of MAS in AOSD. Conclusion The development of MAS in AOSD patient is frequent and hemophagocytie histological evidence could be found in severe cases. When splenomegaly and hypocytomsis present in AOSD patients, bone marrow examination should be done and the level of triglyceride and fibrinogen and activity of NK cells should be measured for early diagnosis.