HScore评分在结缔组织病合并巨噬细胞活化综合征诊断中的应用价值

Assessment the value of HScore in the diagnosis of connective tissue disease with macrophage activation syndrome

目的探讨HScore评分在诊断CTD合并巨噬细胞活化综合征(MAS)中的价值。方法回顾性分析来源于江苏省人民医院风湿免疫科2015年1月至2018年12月CTD合并MAS的住院患者39例,以未合并MAS的SLE 30例、成人斯蒂尔病(ASOD)20例和DM 20例作为对照。2组间率的比较采用χ^2检验或Fisher′s确切概率法检验。采用受试者工作特征曲线(ROC)分析HScore的敏感度和特异度。结果39例CTD-MAS患者中,原发病为SLE 14例(36%),ASOD 9例(23%),未分化CTD 6例(15%),DM 5例(13%),SS 3例(8%),RA 2例(5%)。CTD合并MAS组HScore评分中位数为[191.5(176,389)]显著高于未合并MAS的SLE组[94.5(86,190)](Z=2.51,P<0.05),ASOD组[85.0(66,211)](Z=2.16,P<0.05)和DM组[100.5(74,201)](Z=2.11,P<0.05)。HScore截断值为196.5时,预测CTD合并MAS的敏感度和特异度分别为91.3%和87.4%。分析Hscore的动态变化,CTD-MAS组患者入院早期尚未符合MAS诊断时,HScore评分已显著高于各对照组。当最佳截断值为171时,在入院早期预测CTD患者随后合并MAS的敏感度和特异度分别为83.3%和68.1%。结论HScore提供了一种快速、简便的早期预警和早期识别CTD合并MAS的方法。

Objective To investigate the value of HScore in the diagnosis of connective tissue disease(CTD)combined with macrophage activation syndrome(MAS).Methods Retrospectively analyzed 39 patients with CTD-MAS recruited from First Affiliated Hospital of Nanjing Medical University between January,2015 to December,2018.CTD patients without MAS were used as control,including 30 patients with systemic lupus erythematosus(SLE),20 patients with adult-onset Still's disease(ASOD)and 20 patients with dermatomyositis(DM).Variables were compared between the two group using theχ^2 analysis and Fisher's-exact test.The sensitivity and specificity of HScore were analyzed by receiver operating characteristic(ROC)curve.Results Among 39 CTD-MAS patients,the primary disease consisted of SLE in 14(36%),ASOD in 9(23%),UCTD in 6(15%),dermatomyositis(DM)in 5(13%),Sgören's syndrome in 3(8%)and rheumatoid arthritis(RA)in 2(5%).The median HScore score of 191.5(176,389)in the CTD-MAS group was significantly higher than those in CTD without MAS,including SLE[94.5(86,190)](Z=2.51,P<0.05),ASOD[85.0(66,211)](Z=2.16,P<0.05)and DM[100.5(74,201)](Z=2.11,P<0.05).When the HScore cut-off value was 196.5,the sensitivity and specificity for predicting CTD combined MAS was 91.3%and 87.4%,respectively.Hscore displayed the dynamic changes in CTD-MAS patients.In the early stage of these CTD patients who were not diagnosed as MAS,Hscore was significantly increased,as compared with those of the none-MAS control group.When the optimal cut-off value was 171,the sensitivity and specificity of predicting CTD patients with MAS at early admission were 83.3%and 68.1%,respectively.Conclusion HScore provides a quick and easy method for warning and early identification of CTD combined MAS.