The data of 33 patients with adult-onset still's disease (AOSD)-associated macrophage activation syndrome (MAS) were retrospectively collected from January 2013 to December 2020 in Peking Union Medical College Hospital. Hemophagocytic lymphohistiocytosis (HLH)-2004 criteria, macrophage activation syndrome/juvenile idiopathic arthritis (MS-Score) and hemophagocytic syndrome diagnostic score (HScore) were used to diagnose AOSD-associated MAS, respectively. The time of diagnosis of AOSD-associated MAS by MS-Score was 19.0 (4.5, 31.0) days [M (Q1,Q3)] earlier than by HLH-2004 criteria, and 13.5 (0.5, 21.5) days earlier than by HScore (both P<0.05). The difference was not statistically significant between the time of diagnosis of AOSD-associated MAS by Hscore and by HLH-2004 criteria (P>0.05). There was significant difference among the three criteria (P<0.001). MS-Score can be used to diagnose AOSD-associated MAS earlier than HLH-2004 criteria, while the timeliness of HScore is not certain.
收集2013年1月至2020年12月在北京协和医院出院诊断为成人Still病（AOSD）合并巨噬细胞活化综合征（MAS）的33例患者的临床资料，分析比较分别应用噬血细胞性淋巴组织细胞增多症（HLH）-2004标准、巨噬细胞活化综合征/幼年特发性关节炎评分（MS-Score）和HLH诊断评分（HScore）确诊MAS的时间。结果发现，应用MS-Score确诊MAS的时间［M（Q1，Q3）］比HLH-2004标准提前19.0（4.5，31.0）d，比HScore提前13.5（0.5，21.5）d（均P<0.05）；应用HScore确诊MAS的时间与应用HLH-2004标准差异无统计学意义（P>0.05）；三种诊断标准相比，诊断时间差异有统计学意义（P<0.001）。与HLH-2004标准相比，MS-Score能更早期诊断AOSD合并MAS，而HScore的诊断获益尚未明确。.