Overlapping Autoimmune Syndromes in Patients With Glial Fibrillary Acidic Protein Antibodies.

Xinguang Yang,Tianni Liu,Huacai Yang,Cong Gao,Baikeng Chen,Qingmei Huang,Youming Long,Huiming Xu,Meilin Ding

doi:10.3389/fneur.2018.00251

Abstract

Glial fibrillary acidic protein (GFAP) astrocytopathy, an autoimmune central nervous system disorder with a specific GFAP-IgG, often coexists with other antibodies. The aim of this article was to study overlapping syndromes in autoimmune GFAP astrocytopathy. Antibody was detected by indirect immunofluorescence assay. Patient data were analyzed retrospectively. Thirty patients with positive GFAP-IgG were included, of whom 10 were defined as overlapping syndrome. Four patients with positive aquaporin-4 (AQP4)-IgG, two with N-methyl-d-aspartate receptor-IgG, three with unknown neuronal antibodies, and one with double AQP4 and myelin oligodendrocyte glycoprotein-IgG were identified. GFAP-IgG and other specific antibodies occurred simultaneously at the initial attack in eight patients. The main symptoms included fever, headache, ataxia, psychosis, hypersomnia, dyskinesia, dementia, seizure, myelitis, and optical symptoms. Brain magnetic resonance imaging in four patients revealed characteristic radial enhancing patterns in the white matter. Cortical abnormalities were found in four patients. Other brain abnormalities occurred in the hypothalamus, midbrain, pons, medulla, cerebellum, and meninges. Six patients exhibited lesions in the spinal cord. In a subgroup study, patients with overlapping syndrome were younger at onset than those with non-overlapping syndrome. Overlapping antibodies are common in GFAP astrocytopathy.

Highlights

Aquaporin-4 (AQP4) autoimmunity is associated with the central nervous system (CNS) astrocyte disorder neuromyelitis optica spectrum disorder (NMOSD) [1]
The disease is associated with antibodies against Glial fibrillary acidic protein (GFAP), and the detection of GFAP-IgG is helpful for diagnosis of GFAP astrocytopathy compared with other inflammatory autoimmune disorders of the CNS [2, 3]
A few studies have reported that anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is associated with acute demyelinating encephalomyelitis, myelitis, or NMOSD [6]

Summary

Introduction

Aquaporin-4 (AQP4) autoimmunity is associated with the central nervous system (CNS) astrocyte disorder neuromyelitis optica spectrum disorder (NMOSD) [1]. Glial fibrillary acidic protein (GFAP) astrocytopathy is another severe immune-mediated inflammatory disorder of the CNS, which. The disease is associated with antibodies against GFAP, and the detection of GFAP-IgG is helpful for diagnosis of GFAP astrocytopathy compared with other inflammatory autoimmune disorders of the CNS [2, 3]. Overlapping autoimmune syndromes occur commonly in patients with antibody-mediated inflammatory disorders. NMOSD with positive AQP4 antibody often has a concurrent autoimmune disease, such as Sjögren’s syndrome, myasthenia gravis, or systemic lupus erythematosus [4, 5]. Glial fibrillary acidic protein (GFAP) astrocytopathy, an autoimmune central nervous system disorder with a specific GFAP-IgG, often coexists with other antibodies

Methods

Results

Conclusion