神经精神狼疮109例临床特点及生存分析

The clinical features and survival analysis of 109 patients with neuropsychiatric systemic lupus erythematosus

目的探讨在SLE的不同阶段发病的神经精神狼疮(NPSLE)患者的临床特点、预后情况及影响因素。方法纳入2009年1月至2019年1月在北京大学第一医院住院,确诊神经精神狼疮,并除外其他原因导致的神经精神症状患者进行回顾性研究。通过门诊随访资料及电话随访的方式研究其预后情况,并分析与死亡有关的危险因素。采用t检验(正态分布数据)、非参数检验(非正态分布数据)或χ2检验(计数资料)进行组间比较。使用Kaplan-Meier曲线描述患者的生存情况,使用COX回归分析影响预后的因素。结果共纳入109例NPSLE患者,其中男性13例(11.9%),女性96例(88.1%),发生NPSLE的中位年龄是33岁,以中枢神经系统受累为主(89/109,81.7%),其中最常见的临床类型为头痛、脑血管病及癫痫发作。颅神经病变多见于初发SLE时即有NPSLE的患者,而脑血管病多见于疾病复发时出现NPSLE的患者。初发时即有NPSLE的患者发生SLE后存活时间较复发时出现NPSLE短[(32±26)个月和(197±79)个月,t=2.834,P=0.037]。随访105例患者,自SLE发病和NPSLE发病至末次随访/死亡的中位随访时间分别是118.0(1.4,525.7)个月和53.1(0.4,363.0)个月。共15例(14.3%)患者死亡,发生NPSLE后1~5年的生存率分别为96.2%、94.3%、91.0%、89.9%及88.3%。从SLE发病和NPSLE发生开始,平均生存时间分别为[(180±138)个月和(33±32)个月,t=3.861,P<0.01]。死亡的主要原因包括感染、NPSLE、心血管疾病等。脑血管病是死亡的独立危险因素[RR值(95%CI)=3.413(1.049,11.102),P=0.041]。结论颅神经病变多见于初发时即有NPSLE的患者,脑血管病多见于复发时出现NPSLE的患者。初发SLE时即出现神经精神症状的患者生存期更短。脑血管病是死亡的独立危险因素。

Objective To investigate the clinical characteristics,prognosis,and risk factors for poor prognosis of neuropsychiatric systemic lupus erythematosus(NPSLE).Methods Patients who were diagnosed as NPSLE between January 2009 to January 2019 in Peking University First Hospital were included.Patients with neuro-psychiatric symptoms caused by other reasons such as infection and metabolic disorders were excluded.Patients were retrospectively followed up by telephone or medical records.Continuous variables were compared by student t test or Wilcoxon rank sum test.Quantitative variables were compared by chi-square test.Survival was analyzed by Kaplan-Meier curve.Predictive factors of prognosis was estimated by using Cox regression analysis.Results One hundred and nine NPSLE patients were included.Thirteen(11.9%)were male and 96(88.1%)were female with a median age of 33 years old.Central nervous system involvement was predominant(89/109,81.7%).The most common types were headache,cerebrovascular disease and epilepsy.Cranial neuropathy was the most common type at the initial onset of systemic lupus erythematosus(SLE),while cerebrovascular disease was more common when SLE relapsed.Patients who demonstrated NPSLE at the initiation of SLE had shorter survival time than those who got NPSLE when SLE relapsed[(32±26)months vs(197±79)months,t=2.834,P=0.037].Among the 105 patients with complete followed up data,the follow up time was 118.0(1.4,525.7)months and 53.1(0.4,363.0)months from the onset of SLE and NPSLE,respectively.The mortality rate was 14.3%(15/105).The survival rates of 1-5 years were 96.2%,94.3%,91.0%,89.9% and 88.3%,respectively.The survival time was(180±138)months and(33±32)months,t=3.861,(P<0.01)from the onset of SLE and NPSLE,respectively.The major causes of death were infection,NSPLE and cardiovascular disease.Cerebrovascular disease was the independent risk factor for death[RR=3.413,95%CI(1.049,11.102),P=0.041].Conclusion Cranial neuropathy is the most common type at the initial onset of SLE,while cerebrovascular disease is more common when SLE relapsed.Patients who had NPSLE at the initiation of SLE have shorter survival time than those who got NPSLE when SLE relapsed.Cerebrovascular disease is the independent risk factor of death of NPSLE patients.