188例儿童系统性红斑狼疮5年生存率及预后分析

目的 探讨儿童系统性红斑狼疮(SLE)的临床特点,分析其5年生存率、复发和死亡原因以及影响生存预后的因素,为改善cSLE的预后、指导治疗提供依据。 方法 回顾性收集2009年1月1日至2020年12月31日小儿肾脏风湿免疫科SLE患儿188例的临床资料,并行门诊或电话随访。使用非参数乘积法分析生存率,采用时序检验、比例风险模型行预后因素分析。 结果 188例SLE患儿,男40例,女148例,平均发病年龄(10.64±2.12)岁,发病到确诊中位病程30(15,60)d。最常见的临床表现是发热、皮疹。患儿确诊时最常见合并肾脏损害及血液系统损害。随访期间,73例(38.8%)患儿病情复发,主要原因是依从性差、感染。16例患儿死亡,主要原因是依从性差、多脏器损害、感染、肾功能不全。患儿1、3、5年总体生存率分别为94.1%、92.9%、91.7%。单因素分析显示,心血管系统损害(χ2=4.464,P=0.035)、神经系统损害(χ2=6.545,P=0.011)、血液系统损害(χ2=3.888,P=0.049)、大量蛋白尿(χ2=5.641,P=0.018)、初诊疾病重度活动(χ2=4.679,P=0.031)、诱导治疗疾病无活动(χ2=7.561,P=0.006)、复发(χ2=13.786,P≤0.001)与SLE预后相关,进一步Cox多因素回归分析显示,心血管系统损害(HR=3.361,95%CI:1.251~9.029,P=0.016)、神经系统损害(HR=2.997,95%CI:1.076~8.349,P=0.036)、大量蛋白尿(HR=2.162,95%CI:1.079~4.334,P=0.030)、复发(HR=4.663,95%CI:1.666~13.049,P=0.003)是导致SLE预后不佳的独立危险因素。 结论 本研究SLE患儿主要临床表现以发热、皮疹最为多见,系统损害以肾脏损害、血液系统损害最为多见。合并心血管系统损害、神经系统损害、大量蛋白尿、疾病复发的SLE患儿5年总体预后不佳,治疗时积极控制脏器损害、及时降低蛋白尿、预防疾病复发是提高生存率的关键。

Analysis of 5-year survival rate and prognosis in 188 children with systemic lupus erythematosus

Objective To summarize the clinical characteristics of childhood-onset systemic lupus erythematosus(cSLE), analyze the 5-year survival rate, causes of relapse and death, and factors affecting prognosis, in order to provide a basis for improving the prognosis and guiding the treatment. Methods Clinical data of 188 SLE patients treated during Jan. 1, 2009 and Dec. 31, 2020 were retrospectively collected and the patients were followed up in clinic or with telephone. The survival rate was analyzed using Kaplan-Meier method, and the prognostic factors were analyzed using Log-rank test and cox parametric regression analysis. Results Of the 188 children, 40 were male and 148 were female, mean age of onset were(10.64±2.12)years, median duration of illness from onset to diagnosis were 30(15, 60)days. The most common clinical manifestations were fever and rash. Renal damage and hematologic damage were often observed at diagnosis. During the follow-up, 73 children(38.8%)had relapse, mainly due to poor compliance and infection; 16 children died, mainly due to poor compliance, multiple organ damage, infection, and renal insufficiency. The 1-year, 3-year and 5-year survival rates were 94.1%, 92.9%, and 91.7%, respectively. Univariate analysis showed that cardiovascular system damage(χ2=4.464, P=0.035), neurological system damage(χ2=6.545, P=0.011), hematological system damage(χ2=3.888, P=0.049), massive proteinuria(χ2=5.641, P=0.018), severe activity of the initial disease(χ2=4.679, P=0.031), induction therapy disease inactivity(χ2=7.561, P=0.006), and relapse(χ2=13.786, P≤0.001)were associated with prognosis. Cox parametric regression analysis showed that cardiovascular system damage(HR=3.361, 95%CI: 1.251-9.029, P=0.016), neurological damage(HR=2.997, 95%CI: 1.076-8.349, P=0.036), massive proteinuria(HR=2.162, 95%CI: 1.079-4.334, P=0.030), and relapse(HR=4.663, 95%CI: 1.666-13.049, P=0.003)were independent risk factors of poor prognosis. Conclusion The main clinical manifestations of SLE are fever and rash, and systemic damages are renal damage and hematologic damage. The overall 5-year prognosis for patients complicated with cardiovascular damage, neurological damage, massive proteinuria and with recurrence is poor. Active control of organ damage, timely reduction of proteinuria, and prevention of recurrence are key to improving survival.