Abstract: Objective　To summarize the early clinical characteristics， and laboratory and auxiliary examination characteristics of systemic juvenile idiopathic arthritis （sJIA） with macrophage activation syndrome （MAS），and to screen for the detection indicators with warning of MAS diagnosis to form a scale，in order to achieve the goal of identifying and treating the disease early and reducing mortality. Methods　Retrospective analysis was made on the data of patients with sJIA with MAS in the past 10 years from January 2006 to January 2016. The candidate indicators with evaluation significance for early diagnosis of MAS were selected from the above clinical and auxiliary examination observation indicators. Select the best diagnostic limit value （Cut-off value） by ROC curve method. Multivariate logistic regression was used to analyze the independent risk factors of MAS. The effect was expressed as odds ratio （OR） and 95% confidence interval was calculated. After peer review，the weight score of the above factors was quantified and finally an integral table was formed. Results　In 390 patients with sJIA，141 were with MAS. The clinical manifestations of all the patients included high fever， progressive enlargement of liver，spleen and （or） lymph nodes，and involvement of the blood system. The 19 children with central nervous system involvement had central nervous system function. Single factor Logistic regression and ROC curve analysis showed that there were significant differences in 10 variables between MAS group and severe sJIA group（P<0.05）. Logistic regression analysis results showed that：Fib ≤ 3.11g/L；WBC ≤11.0 × 109/L；SF/ESR ≥ 99.4 and PLT ≤ 260 × 109/L were independent risk factors of MAS. The ROC curve of the model was drawn，and the sensitivity and specificity were 92.42% and 81.20%，respectively. After evaluation and voting by 30 professional experts， the patients were diagnosed for MAS respectively，and the weight values of various clinical manifestations and testing and examination items were scored to form an early warning scale. Conclusion　The clinical manifestations combined with the results of auxiliary examinations and using the form of scoring scale can quickly determine whether severe sJIA is combined with early MAS，which is helpful to advance the diagnosis window，improve the diagnostic rate of MAS，reduce missed diagnosis，and provide reference for prospective research.

Key words: juvenile idiopathic arthritis, macrophage activation syndrome, early stage, warning

摘要： 目的　总结全身型幼年特发性关节炎（sJIA）合并巨噬细胞活化综合征（MAS）的早期临床特征、实验室及辅助检查特点，筛选对诊断MAS有预警作用的检测指标，并量化形成量表，以达到快速对疾病“早识别，早治疗，降低病死率”的目的。方法　回顾性分析2006年1月至2016年1月，北京儿童医院风湿免疫科收治sJIA合并MAS患儿的病例资料，在临床及辅助检查观测指标中筛选出对早期诊断MAS有评估意义的候选指标；通过ROC曲线的方法选择最佳的诊断界限值（Cut-off值）；采用多因素Logistic回归分析MAS独立危险因素，效果以优势比（OR）表示，计算95％置信区间；经过同行评议对上述因素进行权重分值量化并最终形成积分表。结果　390例sJIA患儿，其中141例合并MAS。临床表现全部患儿均有高热、肝脾和（或）淋巴结进行性增大、血液系统受累，中枢神经系统受累患儿19例。单因素Logistic回归及ROC曲线分析结果显示，MAS组和重症sJIA组比较，10个变量存在显著差异（P<0.05）。Logistic回归分析结果显示，Fib ≤3.11g/L、WBC≤11.0×109/L、SF/ESR≥99.4及PLT≤260×109/L为MAS的独立危险因素。绘制模型ROC曲线，灵敏度和特异度分别为92.42%、81.20%。经过30位同行专家评议并投票，分别对上述患儿进行MAS诊断，并对各项临床表现及检验检查项目进行权重值评分，形成早期预警量表。结论　临床表现结合辅助检查结果，利用积分量表的形式，快速判断重症sJIA是否合并早期MAS，对诊断窗口提前，提高MAS诊断率，降低漏诊及前瞻性研究提供参考。

关键词: 幼年特发性关节炎, 巨噬细胞活化, 早期, 预警