川崎病患儿血管内皮细胞抗体检测临床意义分析

Clinical significance of anti-endothelial cell antibodies in children with Kawasaki disease

目的探讨血管内皮细胞抗体(AECA)检测对川崎病(KD)患儿诊断及预后评价。方法2007年8月至2008年5月在广州市儿童医院住院的5岁以下KD患儿55例,采用以人脐静脉内皮细胞和猴骨骼肌为基质的间接免疫荧光技术检测55例急性期KD患儿和43例对照组(发热对照23例,健康对照20名)血清中AECA,并通过比较AECA阴性和阳性组相应的实验室、临床指标,初步评估AECA对KD早期诊断、预后转归的临床应用价值。结果KD患儿中AECA阳性率为40.0%,明显高于健康对照组(5.0%,P=0.004),但与发热对照组差异无统计学意义(17.4%,P=0.053);AECA对KD诊断的灵敏度为40.0%,特异性为88.4%,阳性预测值与阴性预测值则分别为80.0%、53.5%,准确率为60.4%。抗中性粒细胞浆抗体(ANCA)、蛋白酶3抗体(抗-PR3)阳性KD患儿中AECA阳性率显著高于阴性者(P值分别为0.013、0.034)。AECA阳性组与阴性患儿组临床指标、实验室指标差异无统计学意义。AECA阳性组冠状动脉瘤发生率高于阴性组(P<0.05),发生动脉瘤的相对危险度为6.67(95%CI1.2～36.1)。结论AECA检测对KD的早期诊断价值有限,但对冠状动脉瘤的发生具有预警意义。AECA可能与抗-PR3相互协同在KD冠状动脉损伤、进展至动脉瘤过程中起一定的作用。

Objective To investigate the clinical significance of anti-endothelial cell antibodies （AECA） in Chinese children with Kawasaki disease （KD）. Methods By indirect immunofluorenscence assay based on the substrates of human umbilical vein endothelial cells and monkey skeletal muscle sections, AECA was detected in sera from 55 KD children at acute phase and 43 eontrols （including 23 inpatients suffering fever and 20 healthy kids）. Clinical characteristics and laboratory parameters were compared between AECA negative and positive KD patients. Results Twenty-two （40.0%） of the 55 children with KD were found to be positive against endothelial cells, which was higher than that of healthy controls （5.0%, P = 0.004） but did not differ from the fever controls （ 17.4%, P = 0.053）. Sensitivity and specificity of AECA for diagnosis of KD was 40.0% and 88.4% with positive predictive value, negative predictive value and accuracy at 80.0%, 53.5% and 60.4%, respectively. Higher AECA positive rates were observed in anti-neutrophil cytoplasm antibodies （ANCA） positive and proteinase 3 antibodies （anti-PR3） positive KD patients. Compared to AECA negative KD patients, there was no difference found from AECA positive group in clinical characteristics （sex ratio, ages, ages onset and disease duration） and laboratory parameters （complements, immunoglobulin, cholesterol, lipid related parameters, C reactive protein and erythrocyte sedimentation rate）. Probability of coronary aneurysm was higher in AECA positive group than that of negative KD children with relative risk at 6.67 （95% CI：1.2-36.1）. Conclusion The diagnostic value of AECA for Kawasaki disease may be limited, but it is of predictive significance for developing coronary aneurysm. Our data also indicate that AECA might play an important role with anti-PR3 in the pathogenesis of damage of coronary artery leading to coronary aneurysm in Kawasaki disease.