Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study.

Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study.
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DOI:
10.3389/fimmu.2021.621599
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发表时间:
2021
影响因子:
7.3
通讯作者:
Wang Q
Wang Q
中科院分区:
医学2区
文献类型:
--
作者:
Shan W;Yang H;Wang Q

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目的:我国抗体介导的自身免疫性脑炎患者的流行病学特征尚不清楚,需要进行大规模的流行病学调查。方法:多中心回顾性研究。我们收集了1,047例疑似自身免疫性脑炎患者,并最终在中国各中心招募了778例定义的患者。所有患者血清[或脑脊液(CSF)]抗体均为阳性。对2014年1月至2019年1月来自中国22家临床试验机构的人口统计学信息和临床数据进行了审查。结果:共纳入778例自身免疫性脑炎患者。一般而言,男性与女性的比例约为1.2:1。自身免疫性脑炎的主要亚型为NMDAR-AE(61.35%)、LGI-1-AE(20.61%)和GABAbR-AE(12.40%)。从发病年龄特征看,自身免疫性脑炎发病呈“双峰”分布,20岁组和60岁组发病率较高。我们接下来分析了该队列中肿瘤患者的比例。更具体地说,有34名患者患有肿瘤,85名患者肿瘤标志物阳性。在至少1年的随访研究中,81名患者发生复发:52名患有NMDAR-AE(18.2%); 19名患有LGI-1-AE(16.8%); 5名患有GABAbR-AE(9%);以及3名患有CASPR 2-AE。释义:由于中国和西方国家人群之间的人口统计学特征、癌症发病率和遗传特征存在巨大差异,中国自身免疫性脑炎相关的人口统计学特征、性别分布、伴随肿瘤发生率、临床特征和复发特征与西方国家相似,但存在一些微小差异。
Objective: The epidemiological characteristics of patients with antibody-medicated autoimmune encephalitis in China remain unclear, and a large-scale epidemiological survey is necessary. Methods: A multiple-center retrospective study was performed. We collected 1,047 patients with suspected autoimmune encephalitis and ultimately enrolled 778 defined patients across centers in China. All patients were positive for serum [or cerebrospinal fluid (CSF)] antibodies. Demographic information and clinical data from January 2014 to January 2019 from 22 centers in China were reviewed. Results: A total of 778 patients with autoimmune encephalitis were enrolled in the study. In general, the ratio of males to females was ~1.2:1. The main subtypes of autoimmune encephalitis were NMDAR-AE (61.35%), LGI-1-AE (20.61%), and GABAbR-AE (12.40%). According to the characteristics of age of onset, the incidence of autoimmune encephalitis showed a “double peak” distribution entailing a 20-year-old age group and a 60-year-old age group. We next analyzed the proportion of patients with tumors in this cohort. More specifically, there were 34 patients with tumors and 85 with tumor marker positivity. Relapse occurred in 81 patients within at least 1 year's follow up study: 52 with NMDAR-AE (18.2%); 19 with LGI-1-AE (16.8%); 5 with GABAbR-AE (9%); and 3 with CASPR2-AE. Interpretation: Due to the vast differences in demographic features, the incidence of cancer and the genetic characteristics between the populations in China and Western countries, the demographics, sex distribution, concomitant tumor rate, clinical features, and relapse characteristics associated with autoimmune encephalitis in China shows a similar profile with Western countries with some minor differences.
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