目的 探讨儿童自身免疫性胶质纤维酸性蛋白星形胶质细胞病（GFAP-A）的临床特点及治疗预后。方法 回顾性分析2018年6月至2022年7月诊断为GFAP-A患儿的临床资料。结果 5例患儿诊断为GFAP-A，男3例、女2例，起病年龄11.0（5.0~13.5）岁。最常见的首发症状为发热（4例），最常见的神经系统症状和体征分别为头痛（5例）和颈抵抗伴克氏征阳性（5例）。急性期脑脊液检查显示4例白细胞计数及蛋白水平升高，1例蛋白-细胞分离。脑脊液胶质纤维酸性蛋白（GFAP）抗体中位滴度1∶10（1∶10~1∶100），1例合并抗N-甲基-D-天冬氨酸受体（NMDAR）抗体阳性。最常见磁共振成像（MRI）改变为大脑半球柔脑膜强化（4例）。急性期所有患儿首先接受一线免疫治疗（静脉甲泼尼龙冲击联合静脉滴注人免疫球蛋白），4例反应良好，1例脑脊液抗NMDAR抗体阳性者治疗无反应，予利妥昔单抗治疗后好转。随访8.0（4.0~36.0）月，未监测到与该病相关的神经系统后遗症。结论 儿童GFAP-A最常见的临床表现为发热、头痛及脑膜刺激征，MRI以柔脑膜强化最常见，多数患儿一线免疫治疗效果好且在随访期内预后好。

Objective To investigate the clinical characteristics, treatment and prognosis of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in children. Methods The clinical data of children diagnosed with GFAP-A from June 2018 to July 2022 were retrospectively analyzed. Results Five patients were diagnosed with GFAP-A, including 3 boys and 2 girls, and the onset age was 11.0 (5.0-13.5) years. The commonest initial symptom was fever (4 cases), and the commonest neurological symptoms and signs were headache (5 cases) and neck rigidity with positive Kerning sign (5 cases). Cerebrospinal fluid examination (CSF) in acute stage showed elevated white blood cell count and protein level in 4 cases and protein-cell separation in 1 case. The median CSF GFAP antibody titer was 1:10 (1:10-1:100), and 1 patient had combined N-methyl-D-aspartate receptor (NMDAR) antibody. The commonest magnetic resonance imaging (MRI) findings were cerebral leptomeningeal enhancement (4 cases). In acute phase, all children received first-line immunotherapy (high-dose intravenous methylprednisolone combined with intravenous immunoglobulin), and 4 children had a good response. One patient with positive CSF NMDAR antibody had no response to first-line treatment, but improved after rituximab treatment. During a follow-up of 8.0 (4.0-36.0) months, no neurological sequelae associated with the disease were detected. Conclusions The commonest symptoms and signs of GFAP-A in children are fever, headache and meningeal irritation, and leptomeningeal enhancement is the commonest MRI change. Most children have a good response to first-line immunotherapy and have a good prognosis during follow-up.