Translational Neuroimmunology Group, Kids Neuroscience Centre, Children's Hospital at Westmead; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia.
Oxford Autoimmune Neurology Group, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Curr Opin Neurol. 2022 Jun 1;35(3):399-414. doi: 10.1097/WCO.0000000000001048.
Autoimmune encephalitis (AE) refers to immune-mediated neurological syndromes often characterised by the detection of pathogenic autoantibodies in serum and/or cerebrospinal fluid which target extracellular epitopes of neuroglial antigens. There is increasing evidence these autoantibodies directly modulate function of their antigens in vivo. Early treatment with immunotherapy improves outcomes. Yet, these patients commonly exhibit chronic disability. Importantly, optimal therapeutic strategies at onset and during escalation remain poorly understood. In this review of a rapidly emerging field, we evaluate recent studies on larger cohorts, registries, and meta-analyses to highlight existing evidence for contemporary therapeutic approaches in AE.
We highlight acute and long-term treatments used in specific AE syndromes, exemplify how understanding disease pathogenesis can inform precision therapy and outline challenges of defining disability outcomes in AE.
Early first-line immunotherapies, including corticosteroids and plasma exchange, improve outcomes, with emerging evidence showing second-line immunotherapies (especially rituximab) reduce relapse rates. Optimal timing of immunotherapy escalation remains unclear. Routine reporting of outcome measures which incorporate cognitive impairment, fatigue, pain, and mental health will permit more accurate quantification of residual disability and comprehensive comparisons between international multicentre cohorts, and enable future meta-analyses with the aim of developing evidence-based therapeutic guidelines.
自身免疫性脑炎（AE）是指免疫介导的神经系统综合征，常表现为血清和/或脑脊液中存在致病性自身抗体，这些抗体针对神经胶质抗原的细胞外表位。越来越多的证据表明，这些自身抗体在体内直接调节其抗原的功能。早期免疫治疗可改善预后。然而，这些患者通常存在慢性残疾。重要的是，在疾病发作和进展过程中，最佳的治疗策略仍知之甚少。在这篇快速发展的领域的综述中，我们评估了更大队列、登记处和荟萃分析的最新研究，以强调目前 AE 中当代治疗方法的现有证据。
我们强调了特定 AE 综合征中使用的急性和长期治疗方法，举例说明了了解疾病发病机制如何为精准治疗提供信息，并概述了在 AE 中定义残疾结果的挑战。
早期一线免疫疗法，包括皮质类固醇和血浆置换，可改善预后，新出现的证据表明二线免疫疗法（特别是利妥昔单抗）可降低复发率。免疫治疗升级的最佳时机仍不清楚。常规报告包含认知障碍、疲劳、疼痛和心理健康的结局衡量指标，将允许更准确地量化残留残疾，并在国际多中心队列之间进行全面比较，并使未来的荟萃分析能够制定基于证据的治疗指南。
