儿童系统性红斑狼疮210例临床特点及随访研究

Clinical characteristics and follow-up study of 210 children with systemic lupus erythematosus

目的评价儿童系统性红斑狼疮(cSLE)的系统受累、转归等特征及随访情况,探究影响预后的因素。方法收集2016年1月1日至2017年12月31日于首都医科大学附属北京儿童医院首次就诊的系统性红斑狼疮(SLE)患儿,回顾性收集临床表现、各受累系统评估、自身抗体、治疗调整及随访评估等病历资料。应用SPSS 21.0软件进行统计分析及绘图,采用Cox比例风险回归模型等进行影响因素分析。结果共纳入患儿210例,其中男37例,女173例,男女比例为1.0∶4.7;起病年龄为(121.39±30.44)个月。出现皮肤黏膜损害167例(79.5%)、血液系统损害137例(65.2%)、消化系统损害129例(61.4%)、肾脏损害123例(58.6%)、骨骼肌肉系统损害119例(56.7%)、神经系统损害71例(33.8%)、心脏损害68例(32.4%)、呼吸系统损害60例(28.6%)。就诊时90.95%(191/210)的患儿处在中度及以上的疾病活动度,随访1个月时,治疗有效率为76.92%(150/195),随访达1年时,为96.95%(159/164)。补体C3水平高为疾病缓解的保护性因素。糖皮质激素减量至5 mg及以下的患儿共42例,中位时间为40.5(36.0,42.0)个月,发病月龄小、无肾损害为糖皮质激素减停的保护性因素。结论cSLE好发于女性患儿,受累系统多、病情重,经过合理治疗和随访后可于1年后达到疾病缓解或好转。病初补体C3水平高的患儿经治疗后更易于疾病缓解,而发病月龄小、无肾损害的患儿更易于糖皮质激素减停。

Objective To evaluate the systemic involvement,outcome and other disease characteristics of children with systemic lupus erythematosus(cSLE),and to explore the prognostic factors.Methods cSLE treated in Beijing Children′s Hospital,Capital Medical University from January 1,2016 to December 31,2017 were enrolled in this study.Medical records including clinical manifestations and evaluation of affected systems,autoantibodies,treatment adjustment,and follow-up were collected and analyzed retrospectively.SPSS 21.0 was used for statistical analysis and mapping.The prognostic factors were studied by the Cox proportional risk regression model.Results A total of 210 children were included,including 37 males and 173 females,with a male to female ratio of 1.0∶4.7.The average age of onset was(121.39±30.44)months.There were 167(79.5%)patients with skin and mucous membrane damage,137(65.2%)patients with blood system damage,129(61.4%)patients with digestive system damage,123(58.6%)patients with kidney damage,119(56.7%)patients with skeletal and musculoskeletal system damage,71(33.8%)patients with nervous system damage,68(32.4%)patients with heart damage,and 60(28.6%)patients with respiratory system damage.The 90.95%(191/210)of the children enrolled presented moderate or high disease activity at the first visit.The effective rate was 76.92%(150/195)after 1-month follow-up and 96.95%(159/164)after 1-year follow-up.A high level of compliment C3 was a protective factor for disease remission.The glucocorticoid level was declined to 5 mg or less in 42 children,and the median time was 40.5(36.0,42.0)months.Young onset age and no renal damage were protective factors for glucocorticoid reduction.Conclusions cSLE tends to occur in female children with multiple involved systems and severe conditions.After reasonable treatment and follow-up,the disease can be alleviated or improved in one year.A high level of complement C3 at the beginning of disease is conducive to rapid remission of the disease,and the young age of onset and no renal damage is conducive to rapid glucocorticoid reduction.