Abstract: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel autoimmune inflammatory disease of the central nervous system first reported in 2016. The predominant clinical syndrome of GFAP-A is one or more of meningitis, encephalitis and myelitis. The initial symptoms are fever, headache, disturbance of consciousness and positive meningeal irritation. However, the pathogenesis of GFAP-A is unknown and there are no uniform diagnostic criteria for it. At present, it is generally accepted that positive glial fibrillary acidic protein-immunoglobulin G (GFAP-IgG) in serum and cerebrospinal fluid (CSF) is the diagnostic standard. The positive predictive value of GFAP-IgG in CSF is higher than that in serum. Cell-based assay (CBA) and tissue-based assay (TBA) are both recommended methods for detecting GFAP-IgG. GFAP-IgG positive in serum and/or CSF is used to distinguish it from other autoimmune diseases in central nervous system, intracranial inflammatory and neoplastic disease. Most patients respond well to steroid therapy although a small number of patients may leave sequelae or relapse. This paper makes a systematic review on the etiology, pathogenesis, clinical features, diagnosis, differential diagnosis, treatment and prognosis of this disease, hoping that this review can deepen clinicians' understanding of the disease, discover and treat the disease in time.

Key words: Autoimmune diseases of the nervous system, Glial fibrillary acidic protein, Astrocytes, Review

摘要： 自身免疫性胶质纤维酸性蛋白星形胶质细胞病系2016年提出并命名的一种新型中枢神经系统自身免疫性疾病。临床主要表现为累及脑膜、脑实质或脊髓的炎症或者上述各部位炎症的组合。通常以发热、头痛、意识障碍和脑膜刺激征阳性为首发症状。发病机制尚不清楚，尚无统一的诊断标准，主要以血清和（或）脑脊液抗胶质纤维酸性蛋白抗体（GFAP-IgG）阳性为诊断标准，且脑脊液GFAP-IgG阳性预测值高于血清，推荐基于细胞的检测和基于组织的检测为检测GFAP-IgG的有效方法。应注意与其他发生于中枢神经系统的自身免疫性疾病、颅内炎症性和肿瘤性病变相鉴别。大多数患者糖皮质激素治疗反应良好，少数可遗留后遗症或复发。本文综述自身免疫性胶质纤维酸性蛋白星形胶质细胞病病因及发病机制、临床特征、诊断、鉴别诊断、治疗及预后，以期加深对疾病的认识，早期诊断、及时治疗。

关键词: 神经系统自身免疫疾病, 神经胶质原纤维酸性蛋白质, 星形细胞, 综述