自身免疫性脑炎（AE）是一种中枢神经系统炎症性疾病，其特征是产生多种针对神经元蛋白的自身免疫抗体。 AE 的发病机制仍然难以捉摸。越来越多的证据表明淋巴细胞，特别是 B 和 T 淋巴细胞，在 AE 的发展中发挥着不可或缺的作用。在过去的二十年中，自身免疫神经抗体已成为 AE 诊断的中心舞台。最近，越来越多的证据强调了 T 淋巴细胞在 AE 发病中的重要性。 CD4 T 细胞被认为通过分泌相关细胞因子来影响疾病进展，而 CD8 T 细胞发挥细胞毒性作用，主要在副肿瘤 AE 患者中对神经元造成不可逆的损伤。传统上，AE 的一线治疗包括静脉注射类固醇、静脉注射免疫球蛋白和血浆置换以去除致病性自身抗体。然而，少数患者对传统的一线治疗方案不敏感，并且会出现疾病复发，这种情况被称为难治性 AE。近年来，针对AE患者致病性淋巴细胞的新疗法，如利妥昔单抗或CAAR-T，为难治性AE提供了新的治疗选择。本综述旨在描述目前关于 B 和 T 淋巴细胞在 AE 病理生理学中的功能的知识，并总结和更新治疗该疾病的免疫治疗方案。版权所有 © 2024。由 Elsevier Inc. 出版。

Autoimmune encephalitis (AE) is an inflammatory disease of the central nervous system characterized by the production of various autoimmune antibodies targeting neuronal proteins. The pathogenesis of AE remains elusive. Accumulating evidence suggests that lymphocytes, particularly B and T lymphocytes, play an integral role in the development of AE. In the last two decades, autoimmune neural antibodies have taken center stage in diagnosing AE. Recently, increasing evidence has highlighted the importance of T lymphocytes in the onset of AE. CD4+ T cells are thought to influence disease progression by secreting associated cytokines, whereas CD8+ T cells exert a cytotoxic role, causing irreversible damage to neurons mainly in patients with paraneoplastic AE. Conventionally, the first-line treatments for AE include intravenous steroids, intravenous immunoglobulin, and plasma exchange to remove pathogenic autoantibodies. However, a minority of patients are insensitive to conventional first-line treatment protocols and suffer from disease relapse, a condition referred to as refractory AE. In recent years, new treatments, such as rituximab or CAAR-T, which target pathogenic lymphocytes in patients with AE, have offered new therapeutic options for refractory AE. This review aims to describe the current knowledge about the function of B and T lymphocytes in the pathophysiology of AE and to summarize and update the immunotherapy options for treating this disease.Copyright © 2024. Published by Elsevier Inc.