Research progress of autoimmune glial fibrillary acidic protein astrocytopathy

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.

Keywords

• autoimmune diseases of the nervous system
• glial fibrillary acidic protein
• astrocytes
• review