自身免疫性胶质纤维酸性蛋白星形胶质细胞病研究进展

Research progress of autoimmune glial fibrillary acidic protein astrocytopathy

自身免疫性胶质纤维酸性蛋白星形胶质细胞病(GFAP-A)是一种新型中枢神经系统自身免疫性疾病。该病可累及大脑、小脑、视神经和脊髓的任一区域,以脑炎、脑膜脑炎、脑脊髓炎最为常见。患者常以头痛、发热、意识障碍、自主神经功能障碍、视力损害等为表现,主要以脑脊液和血清中抗胶质纤维酸性蛋白抗体阳性作为诊断依据。该病对糖皮质激素敏感,70%的患者予大剂量激素冲击治疗预后良好,约1/5的患者遗留神经系统后遗症,需长期予免疫抑制剂治疗。现通过综合分析国内外研究进展,总结GFAP-A可能的病因、发病机制、临床特征、诊断与鉴别诊断、治疗及预后,以期加深对该病的认识,为临床的诊断和治疗提供参考。

Autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A)is a novel autoimmune disease of the central nervous system.The disease can affect any region of the cerebrum,cerebellum,optic nerve,and spinal cord,with encephalitis,meningoencephalitis and encephalomyelitis being the most common.Patients usually present with headache,fever,disturbance of consciousness,autonomic dysfunction,and visual impairment,and the diagnosis is mainly based on positive anti-glial fibrillary acidic protein antibodies in cerebrospinal fluid and serum.The disease is sensitive to glucocorticoids,with 70%of patients responding well to high-dose glucocorticoid pulse therapy and having a good prognosis.About one in five patients have residual nervous system sequelae,requiring long-term immunosuppressive therapy.This paper reviews the etiology,pathogenesis,clinical features,diagnosis and differential diagnosis,treatment and prognosis of GFAP-A by analyzing relevant studies at home and abroad,in order to deepen the understanding of this disease and provide references for its clinical diagnosis and treatment.