The central nervous system involvement of patients with microscopic polyangiitis
10.3760/cma.j.cn141217-20210201-00048
- VernacularTitle:显微镜下多血管炎中枢神经系统受累临床特征分析
- Author:
Zhihong TIAN
1
;
Yin ZHAO
;
Yanan WU
;
Fumin QI
;
Ruoming WANG
;
Wei WEI
Author Information
1. 天津市第三中心医院肾病免疫科 天津市重症疾病体外生命支持重点实验室 天津市人工细胞工程技术研究中心 天津市肝胆疾病研究所，天津 300170
- Keywords:
Microscopic polyangiitis;
Vasculitis, central nervous system;
Clinical features
- From:
Chinese Journal of Rheumatology
2023;27(3):178-183,c3-2
- CountryChina
- Language:Chinese
-
Abstract:
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. Results:①29 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 ( χ2=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.
