幼年特发性关节炎全身型早期诊断标准探讨

摘要

目的 通过回顾性分析与比较幼年特发性关节炎全身型(systemic onset juvenile idiopathic arthritis,SOJIA)与其他疾病引起的发热待查患儿的临床特点及实验室检查结果,来获得鉴别诊断的临界值,以提高早期诊断SOJIA的准确性.方法 收集不明原因发热患儿共124例,记录患儿持续发热达2～4周时的临床特点和实验室检查结果,并随访1年以上.对资料完整的病例采用SPSS16.0软件进行单因素分析,对有统计学意义的单因素进行受试者工作特征曲线(ROC)分析,然后对有意义的临床特点和实验室检查结果进行联合分析.结果 诊断明确且资料完整者共96例,其中33例为SOJIA,19例为其他自身免疫性疾病,25例为血液/肿瘤疾病,19例为感染性疾病.当血清铁蛋白(SF)≥545.75 ng/ml时,可以将SOJIA与其他自身免疫性疾病区分的灵敏度为97.0％,特异度为100％,若以出现皮疹或关节肿/痛加上血小板计数≥217×109/L或C3≥1.275 g/L作为标准,其区分SOJIA和血液肿瘤疾病的灵敏度为100％,特异度为96％.若以SF≥441.7ng/ml加上出现皮疹或关节肿/痛,那么其区分SOJIA和感染性疾病的灵敏度和特异度均可达到100％.结论 结合发热待查患儿的临床特点及实验室检查结果可提高早期诊断SOJIA的准确性.%Objective To identify the cut-off points for differential diagnosis to increase the accuracy of early diagnosis for systemic onset juvenile idiopathic arthritis (SOJIA) by retrospectively analysis on the patients with SOJIA and the patients with fever of unknown origin. Methods One hundred and twenty-four patients with fever of unknown origin were enrolled in this study. The clinical features and the results of laboratory tests of the patients with persistent fever for 2 to 4 weeks were recorded completely and they were then followed up for more than one year. The data were analysed by single factor analysis using SPSS16.0. The areas under the ROC curve of the measurement data which had statistical significance were calculated. Then the significant clinical features and the results of laboratory tests were analyzed and integrated. Results There were 96 patients who had definite diagnosis and complete data. Among them, 33 patients were diagnosed with SOJIA, 19 with the other autoimmune diseases, 25 with hematologic or oncologic diseases and 19 with infectious diseases. When serum ferritin (SF) higher than 545.75 ng/ml was taken as the cut-off point, the sensitivity and specificity of differentiating SOJIA from the other autoimmune diseases was 97% and 100%, respectively. If rash or joints swelling/pain in addition of the platelet count higher than 217×109/L or the value of C3 higher than 1.275 g/L were taken as the standard, the sensitivity and specificity of differentiating SOJIA from hematologic or oncologic diseases were 100% and 96%, respectively. When rash or joints swelling/pain in addition of SF higher than 441.7 ng/ml were taken as standard, both the sensitivity and specificity of differentiating SOJIA from the infectious diseases were all 100%. Conclusions Combination of the clinical features with laboratory test results could increase the accuracy of early diagnosis for systemic onset juvenile idiopathic arthritis.