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胶质纤维酸性蛋白作为视神经脊髓炎谱系障碍的生物标志物:当前综述

Glial fibrillary acidic protein as a biomarker in neuromyelitis optica spectrum disorder: a current review.

作者信息

Schindler Patrick, Aktas Orhan, Ringelstein Marius, Wildemann Brigitte, Jarius Sven, Paul Friedemann, Ruprecht Klemens

机构信息

Experimental and Clinical Research Center, A Cooperation between the Max Delbrück Center for Molecular Medicine in the Helmholtz Association and the Charité Universitätsmedizin Berlin, Berlin, Germany.

Department of Neurology, Charité Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.

出版信息

Expert Rev Clin Immunol. 2023 Jan;19(1):71-91. doi: 10.1080/1744666X.2023.2148657. Epub 2022 Nov 30.

Abstract

INTRODUCTION

Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing, often debilitating neuroinflammatory disease, whose predominant clinical manifestations are longitudinally extensive transverse myelitis and optic neuritis. About 80% of the patients with an NMOSD phenotype have pathogenic autoantibodies against the astrocyte water channel aquaporin-4 (AQP4-IgG). While therapeutic options for NMOSD have greatly expanded in recent years, well-established biomarkers for prognosis or treatment response are still lacking. Glial fibrillary acidic protein (GFAP) is mainly expressed in astrocytes and can be detected in cerebrospinal fluid (CSF) and blood of patients with NMOSD.

AREAS COVERED

Here, we comprehensively review the current knowledge on GFAP as a biomarker in NMOSD.

EXPERT OPINION

In patients with AQP4-IgG NMOSD, GFAP levels are elevated in CSF and serum during acute attacks and correlate with disability, consistent with the pathophysiology of this antibody-mediated astrocytopathy. Serum GFAP levels tend to be higher in AQP4-IgG NMOSD than in its differential diagnoses, multiple sclerosis, and myelin oligodendrocyte antibody-associated disease. Importantly, serum GFAP levels in AQP4-IgG NMOSD during remission may be predictive of future disease activity. Serial serum GFAP measurements are emerging as a biomarker to monitor disease activity in AQP4-IgG NMOSD and could have the potential for application in clinical practice.

摘要

引言

视神经脊髓炎谱系障碍(NMOSD)是一种复发性、常导致功能障碍的神经炎症性疾病,其主要临床表现为长节段横贯性脊髓炎和视神经炎。约80%具有NMOSD表型的患者存在针对星形胶质细胞水通道蛋白4(AQP4-IgG)的致病性自身抗体。尽管近年来NMOSD的治疗选择有了很大扩展,但仍缺乏用于预后或治疗反应的成熟生物标志物。胶质纤维酸性蛋白(GFAP)主要在星形胶质细胞中表达,在NMOSD患者的脑脊液(CSF)和血液中均可检测到。

涵盖领域

在此,我们全面综述了目前关于GFAP作为NMOSD生物标志物的知识。

专家观点

在AQP4-IgG NMOSD患者中,急性发作期间脑脊液和血清中的GFAP水平升高,且与残疾程度相关,这与这种抗体介导的星形细胞病的病理生理学一致。AQP4-IgG NMOSD患者血清GFAP水平往往高于其鉴别诊断疾病、多发性硬化症和髓鞘少突胶质细胞抗体相关疾病。重要的是,缓解期AQP4-IgG NMOSD患者的血清GFAP水平可能预测未来疾病活动。连续测量血清GFAP正成为监测AQP4-IgG NMOSD疾病活动的一种生物标志物,并且有可能应用于临床实践。

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