2019欧洲抗风湿病联盟/美国风湿病学会系统性红斑狼疮分类标准在儿童患者中的应用价值  

Clinical value of 2019 European League Against Rheumatism/American College of Rheumatology classification criteria in childhood-onset systemic lupus erythematosus

作　　者：程桑 曹兰芳[1] Cheng Sang;Cao Lanfang(Department of Pediatrics,Renji Hospital,Shanghai Jiaotong University School Medicine,Shanghai 200001,China)

机构地区：[1]上海交通大学医学院附属仁济医院儿科,200001

出　　处：《中华实用儿科临床杂志》2021年第17期1305-1309,共5页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的比较2019欧洲抗风湿病联盟/美国风湿病学会(EULAR/ACR)系统性红斑狼疮(SLE)分类标准与ACR1997、系统性狼疮国际合作组(SLICC)2012标准在儿童SLE中的诊断价值。方法回顾性分析2013年1月至2017年5月在上海交通大学医学院附属仁济医院儿科确诊的182例SLE患儿及163例非SLE结缔组织病患儿的临床资料。按照ACR1997、SLICC2012和2019EULAR/ACR的SLE分类标准对其临床表现及实验室数据进行填表,统计标准诊断率。结果1.SLE组与非SLE组患儿发热(21.4%比8.0%)、皮肤损害(54.9%比31.9%)、非瘢痕性脱发(3.8%比0)、肾损害(41.2%比5.5%)、神经系统损害(7.7%比1.8%)、血液系统异常「白细胞减少(32.4%比1.8%)、血小板减少(31.9%比0)」、补体降低(83.5%比12.9%)、抗核抗体(98.4%比23.3%)、抗双链DNA抗体(94.0%比8.6%)、抗Sm抗体(19.2%比0)、抗磷脂抗体(16.5%比3.7%)阳性率比较差异均有统计学意义(均P<0.05);但口腔溃疡、滑膜炎、浆膜炎、直接抗人球蛋白试验(Coombs试验)阳性比较差异均无统计学意义(均P>0.05)。2.ACR1997、SLICC2012、2019EULAR/ACR分类标准的敏感性分别为67.0%(122/182例)、95.6%(174/182例)、97.8%(178/182例)(P<0.001),特异性分别为99.4%(162/163例)、98.2%(160/163例)、94.5%(154/163例)(P=0.016);在准确度上,三者分别为82.3%(284/345例)、96.8%(334/345例)、96.2%(332/345例),差异有统计学意义(P<0.001)。3.临床确诊SLE患儿中,仅120例(65.9%)同时符合3个分类标准;仅符合2019EULAR/ACR分类标准者共8例,其中7例为单脏器受累;不符合2019EULAR/ACR标准共4例,其中3例为抗核抗体阴性。4.SLICC2012和2019EULAR/ACR标准对重要脏器损害的SLE患者敏感性较ACR1997高;2019EULAR/ACR分类标准评分与疾病活动度呈正相关(R2=0.451,P<0.001)。结论2019EULAR/ACR分类标准对儿童SLE患者的敏感性优于ACR1997和SLICC2012,有利于疾病早期诊断及单脏器、重要脏器受累患者的识别,虽然特异性略低,但同样值得Objective To compare the performance among the American College of Rheumatology(ACR)1997,the Systemic Lupus International Collaborating Clinics(SLICC)2012 and the 2019 European League Against Rheumatism(EULAR)/ACR criteria,in a childhood-onset systemic lupus erythematosus(CSLE)cohort.Methods A medical chart review study was conducted of 182 cases of SLE patients and 163 controls with defined rheumatic diseases in pediatrics department of Renji Hospital,Shanghai Jiaotong University School Medicine,from January 2013 to May 2017,to establish each ACR1997,SLICC2012 and 2019EULAR/ACR criterion.The performance of the three criteria was statistically analyzed.Results(1)Comparing the patients with SLE and controls,the difference in fever(21.4%vs.8.0%),skin lesions(54.9%vs.31.9%),nonscarring alopecia(3.8%vs.0),renal disorder(41.2%vs.5.5%),neurologic disorder(7.7%vs.1.8%),hematologic disorder[leukopenia(32.4%vs.1.8%),thrombocytopenia(31.9%vs.0)],low complement(83.5%vs.12.9%),anti-nuclear antibody(98.4%vs.23.3%),anti-dsDNA antibody(94.0%vs.8.6%),anti-Sm antibody(19.2%vs.0%),and antiphospholipid antibodies(16.5%vs.3.7%)had statistical significance(all P<0.05).But the difference in oral ulcers,synovitis,serositis and positive Coombs test had no statistical significance(all P>0.05).(2)Sensitivities of ACR1997,SLICC2012 and 2019EULAR/ACR criteria were 67.0%(122/182 cases),95.6%(174/182 cases)and 97.8%(178/182 cases)(P<0.001),with specificities 99.4%(162/163 cases),98.2%(160/163 cases)and 94.5%(154/163 cases)(P=0.016),respectively.In terms of accuracy,the three classifications were 82.3%(284/345 cases),96.8%(334/345 cases)and 96.2%(332/345 cases),respectively,the difference was statistically significant(P<0.001).(3)Only 120 cases(65.9%)of patients with SLE met all 3 criteria.Eight cases of SLE patients who only met the 2019EULAR/ACR criteria exhibit high rate of single organ involvement(7 cases).Four cases of SLE patients were missed by the 2019EULAR/ACR,3 cases of which were antinuclear antibody negative.(4)The SLICC2012 and 20

关 键 词：系统性红斑狼疮 儿童 分类标准 

分 类 号：R725.9[医药卫生—儿科]