托珠单抗在热性感染相关性癫痫综合征中的应用现状
收稿日期: 2025-05-07
录用日期: 2025-07-23
网络出版日期: 2026-03-06
Clinical application of tocilizumab in febrile infection-related epilepsy syndrome
Received date: 2025-05-07
Accepted date: 2025-07-23
Online published: 2026-03-06
热性感染相关性癫痫综合征（FIRES）是一种罕见的严重癫痫性脑病。FIRES主要累及学龄期儿童，表现为前驱发热感染后迅速进展为难治性癫痫持续状态，病程呈双相性。该病预后差，急性期病死率约10%，慢性期幸存者常遗留难治性癫痫及认知障碍。FIRES发病机制尚不明确，以白细胞介素-6（IL-6）为代表的细胞因子参与的神经炎症可能起关键作用。托珠单抗（tocilizumab）是一种人源化的IL-6受体单克隆抗体，可通过抑制IL-6信号通路发挥抗炎作用，对于常规抗癫痫发作药物、一线免疫治疗及生酮饮食等无效的FIRES患者，托珠单抗目前被推荐用于二线免疫治疗选项。本文就托珠单抗在FIRES中应用的背景、机制、现状、安全性及挑战等方面进行综述，以帮助临床医师了解该领域的最新进展。
关键词： 热性感染相关性癫痫综合征; 难治性癫痫持续状态; 白细胞介素-6; 托珠单抗
陈哲 , 洪思琦 . 托珠单抗在热性感染相关性癫痫综合征中的应用现状[J]. 临床儿科杂志, 2026 , 44(3) : 264 -269 . DOI: 10.12372/jcp.2026.25e0509
Febrile infection-related epilepsy syndrome (FIRES) is a rare and catastrophic epileptic encephalopathy that predominantly affects school-aged children.Characterized by a biphasic clinical course, FIRES manifests as rapid progression to refractory status epilepticus following a preceding febrile infection. The prognosis of FIRES is dismal: the acute-phase mortality rate is approximately 10%, and survivors often develop chronic refractory epilepsy and cognitive impairment. The pathogenesis of FIRES remains unclear, but neuroinflammation involving cytokines such as IL-6 may play a key role. Tocilizumab, a humanized monoclonal antibody targeting the IL-6 receptor, exerts anti-inflammatory effects by blocking IL-6 signaling. For FIRES patients unresponsive to conventional anti-seizure medications (ASMs), first-line immunotherapies, or ketogenic diet, tocilizumab is currently recommended as a second-line immunotherapeutic option. This review summarizes the background, mechanisms, clinical applications, safety profile, and challenges associated with tocilizumab in FIRES management, aiming to update clinicians on the latest advances in this field..
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