显微镜下多血管炎中枢神经系统受累临床特征分析

The central nervous system involvement of patients with microscopic polyangiitis

目的探究显微镜下多血管炎(MPA)中枢神经系统(CNS)受累的临床特点与预后。方法回顾性分析2010年1月1日至2019年11月1日就诊于天津医科大学总医院的138例MPA患者临床资料。根据有无CNS受累分为2组,采用Kaplan-Meier分析中枢神经系统受累对MPA生存率的影响,Logistic回归模型分析进行危险因素分析,以P<0.05为差异有统计学意义。结果①138例MPA患者中枢神经系统受累29例(21.0%),男性13例(44.8%),女性16例(55.2%)。20例(69.0%)发生在诊断时,9例(31.0%)发生在诊断后。②临床表现为运动功能障碍14例(48.3%),感觉功能障碍10例(34.5%),失语9例(31%),头痛8例(27.6%),意识障碍7例(24.1%),吞咽困难、饮水呛咳4例(13.8%),颅神经受累3例(10.3%)。头颅影像表现有脑梗死、脑出血、脑梗死合并脑出血、硬脑膜增厚并线样强化。5例行脑脊液检查,1例脑脊液压力升高,1例脑脊液白蛋白升高,5例脑脊液LDH升高。③中枢神经系统受累患者中18例采用激素联合环磷酰胺治疗。6例出现复发,4例为再次出现脑梗死。④CNS受累组中位生存时间为55个月[95%CI=(14.215,95.785)],无CNS受累(N-CNS)组的中位生存时间为86个月[95%CI=(24.378,147.622)]。Kaplan-Meier生存分析显示2组间生存率差异无统计学意义(χ^(2)=0.07,P=0.794)。结论MPA中枢神经系统受累并不少见,其临床表现复杂多样,运动功能障碍最常见。最常见的影像表现是脑梗死,并且为多灶性脑梗死。MPA中枢神经系统受累与死亡率无相关关系。

Objective To explore the clinical features and prognosis of central nervous system involvement in patients with microscopic polyangiitis(MPA).Methods We retrospectively investigated the clinical data of 138 MPA patients hospitalized with MPA in Tianjin Medical University General Hospital from January 1,2010 to November 1,2019.Patients were divided into two groups according to whether they had the central nervous system(CNS)involvement or not and then Kaplan-Meier survival curve was used to analyze the survival rate between the two groups,Logistic regression model analysis was adopted to analyze risk factors,and P<0.05 was considered statistically significant.Results①29 patients(21.0%)among the 138 MPA had CNS-affected,including 13(44.8%)males and 16(55.2%)females.CNS involvement was present at the diagnosis of MPA in 20 cases(69.0%)and after the diagnosis of MPA in 9 cases(31.0%).②The clinical manifestations were motor impairment in 14 cases(48.3%),sensory impairment in 10 cases(34.5%),speech loss in 9 cases(31.0%),headache in 8 cases(27.6%),consciousness disorder in 7 cases(24.1%),dysphagia and bucking in 4 cases(13.8%),cranial nerves involvement in 3 cases(10.3%).The imaging manifestations of the head included infarction,hemorrhage,infarction with hemorrhage and linear dural thickening.Five patients received lumbar puncture.One patient showed elevation of cerebrospinal fluid pressure,1 patient had elevated protein and 5 patients showed elevation of LDH.③Eighteen patients received glucocortoid combined with cyclophosphamide.CNS symptoms recurred in 6 patients,four patients had recurrent cerebral infarction.④Median survival time was 55 months in the CNS affected group[95%CI=(14.215,95.785)]and 86 months in the N-CNS group[95%CI=(24.378,147.622)].Kaplan-Meier survival curve showed that there was no significant difference in survival rate between the two groups(χ^(2)=0.07,P=0.794).Conclusion The central nervous system involvement of microscopic polyangiitis is not uncommon.The clinical manifestations are various,with motor impairment the most.The most common imaging manifestation is cerebral infarction and the patients mainly presenteas multiple cerebral infarction.However,the CNS involvement of microscopic polyangiitis is not associated with mortality.