儿童系统性红斑狼疮并发血栓的临床特点及随访研究

Clinical characteristics and follow-up of thrombosis in pediatric patients with systemic lupus erythematosus

目的探讨儿童SLE合并血栓的临床特征及转归情况。方法收集2006年1月至2019年12月在北京儿童医院确诊SLE合并动脉或静脉血栓的住院患者,分析其临床特点及转归。应用SPSS 25.0软件对数据进行统计学分析,组间差异比较采用t检验或χ2检验,应用Kaplan-Meier生存曲线对SLE患儿发生血栓终点事件的时间进行分析。结果2006年1月到2019年12月,共收治1395例新发SLE患者,其中血栓病例共27例,占所有SLE患者的1.9%。诊断SLE到发生血栓的中位病程为20 d(诊断前49 d至诊断后1年)。27例患儿中,22例(81%)患者合并肾脏受累,诊断SLE时和发生血栓时的SLEDAI评分为(14±6)分和(11±4)分(t=2.547,P=0.017)。30%(8/27)的患儿发生血栓时无明显血栓临床表现。患儿明确诊断血栓后均接受规范抗凝治疗。其中6例在随访过程中因原发病重放弃治疗。21例均进行规律随访,随访时间1个月至3年;12例(44%)患儿血栓溶解再通,再通时间16 d至1年,9例(33%)血栓好转缩小,原发病稳定。结论SLE患儿发生血栓并不罕见,且部分患儿无明显血栓相关的临床表现。有肾脏受累的患儿更需警惕血栓发生。早期发现积极治疗是改善儿童SLE血栓预后的关键。

Objective To investigate the clinical characteristics and follow-up of thrombosis of pediatric patients with systemic lupus erythematosus(SLE).Methods In this retrospective study,inpatients who were diagnosed in Beijing Children's Hospital with SLE complicated with arterial or venous thrombosis from January 2006 to December 2019 were collected,the clinical characteristics and outcomes were analyzed.Statistical product and Service solutions(SPSS)25.0 was used for statistical analysis.T test orχ2 test(counting data)was used to compare the differences between groups,and Kaplan-Meier survival curve was used to analyze the time of thrombus endpoint events in lupus children.Results A total of 1395 newly diagnosed SLE patients were admitted.Twenty-seven cases were diagnosed with thrombosis,accounting for 1.9%of all the lupus patients.The median course from diagnosis to thrombosis was 20 days(49 days before diagnosis to 1 year after dia-gnosis).Among the 27 patients,22(81%)cases had renal involvement.The mean SLE disease activity index(SLEDAI)score was(14±6)and(11±4)at the diagnosis of lupus and at onset of thrombosis,respectively(t=2.547,P=0.017).30%(8/27)of the patients had no apparent clinical manifestations of thrombosis.The patients received standard anticoagulant therapy after the diagnosis of thrombosis.During follow-up,6 patients stopped taking medications due to the severity of the primary disease.Twenty-one patients were followed up regularly for 1-3 years.Thrombosis disappeared in 12 cases(44%),thrombolysis time ranged from 16 days to 1 year.Thrombosis were getting smaller in 9 cases(33%).And the disease was stable during follow up.Conclusion Thrombosis is not rare in pediatric patients with systemic lupus erythematosus patients.Some patients do not have apparent clinical manifestations related to thrombus.Pediatricians should be alert to patients with renal involvement need to be more vigilant for thrombosis.Early detection and active treatment are the keys to improve the prognosis of thrombosis in pediatric SLE patients.