Are the 2016 EULAR/ACR/PRONTO classification criteria for macrophage activation syndrome applicable to patients with adult-onset Still's disease?
Are the 2016 EULAR/ACR/PRONTO classification criteria for macrophage activation syndrome applicable to patients with adult-onset Still's disease?
复制标题
2016 年 EULAR/ACR/PRONTO 巨噬细胞活化综合征分类标准是否适用于成人斯蒂尔病患者？
DOI：
10.1007/s00296-018-4114-1
复制
发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Koarada S
中科院分区：
文献类型：
--
作者：
Tada Y;Inokuchi S;Maruyama A;Suematsu R;Sakai M;Sadanaga Y;Ono N;Arinobu Y;Koarada S
本研究的目的是确定2016年欧洲抗风湿病联盟/美国风湿病学会/儿科风湿病国际试验组织关于巨噬细胞激活综合征(MAS)并发系统性幼年特发性关节炎(SJIA)的分类标准是否可用于识别成人起病斯蒂尔病(AOSD)患者的MAS。我们利用76例合并和不合并大脑中动脉的AOSD患者的实验室数据，分析了SJIA标准和附加实验室措施中2016年MAS的集体和个体构成要素区分有( 16)和不( 60)大脑中动脉的AOSD患者的能力。根据受试者操作特征曲线计算确定敏感性、特异性和预测值的临界值，并评估AOSD中MAS的改良分类标准。根据实验室数据，SJIA分类标准中2016 MAS对AOSD患者合并和不合并MAS的总体敏感性为100%，特异性为70.0%，阳性预测值为47.1%，阴性预测值为100%。在单项标准中，甘油三酯(46.7%)和铁蛋白(15.0%)对AOSD患者MAS的敏感性(46.7%)和特异性(15.0%)尤其低。通过排除甘油三酯和改变2016年SJIA MAS分类中其他标准的临界值，AOSD患者MAS分类的敏感性和特异性分别提高到100%和93%。2016 SJIA MAS分级标准对AOSD患者MAS诊断的敏感性高于SJIA患者，但特异性低于SJIA患者。
The objectives of this study are to determine whether the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (SJIA) can be used to identify MAS in patients with adult-onset Still’s disease (AOSD). Using laboratory data from 76 AOSD patients with and without MAS, we analyzed the ability of the collective and individual constitutive elements of the 2016 MAS in SJIA criteria and additional laboratory measures to discriminate between AOSD patients with (n= 16) and without (n= 60) MAS. Cutoff values to determine the sensitivity, specificity, and predictive values were calculated from receiver operating characteristic curves, and modified classification criteria for MAS in AOSD were evaluated. The 2016 MAS in SJIA classification criteria had an overall sensitivity of 100%, specificity of 70.0%, positive predictive value of 47.1%, and negative predictive value of 100% to discriminate between AOSD patients with and without MAS based on laboratory data. Among the individual criteria, the sensitivity of triglycerides (46.7%) and the specificity of ferritin (15.0%) for MAS in AOSD were particularly low. The sensitivity and specificity for classifying MAS in AOSD patients were increased to 100 and 93%, respectively, by excluding triglycerides and changing the cutoff values for other criteria in the 2016 MAS in SJIA classification. The 2016 classification criteria for MAS in SJIA had higher sensitivity but lower specificity to identify MAS in AOSD patients compared with SJIA patients.