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自身抗体在炎性神经疾病中的诊断效用

Diagnostic Utility of Auto Antibodies in Inflammatory Nerve Disorders.

作者信息

Emilien Delmont, Hugh Willison

机构信息

Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, United Kingdom.

Referral centre for ALS and Neuromuscular diseases, hospital La Timone, Marseille, France.

出版信息

J Neuromuscul Dis. 2015 Jun 4;2(2):107-112. doi: 10.3233/JND-150078.

Abstract

A wide range of autoantibodies have been described in immune-mediated nerve disorders that target glycans borne by glycolipids and glycoproteins enriched in the peripheral nerves. Their use as diagnostic biomarkers is very widespread, despite some limitations on sensitivity and specificity, and the lack of standardized assays and access to quality assurance schemes. Although many methods have been applied to measurement, ELISA, in the form of commercial kits or in-house assays, still remains the most widely available and convenient assay methodology.Some antibodies have a particularly robust and widely appreciated clinical significance. Thus, the anti-MAG IgM antibodies that are found in IgM paraprotein related neuropathies define a relatively uniform clinical and prognostic phenotype. IgG antibodies against gangliosides GM1 and GD1a are strongly associated with motor axonal variants of Guillain-Barré syndrome, and anti-GQ1b with Miller Fisher syndrome. In other chronic neuropathies, antibodies against disialylated gangliosides including GD1b and GD3 are detected in ataxic neuropathies, usually associated with an IgM paraprotein, and antibodies against GM1 and the complex GM1:GalC are frequently found in multifocal motor neuropathy. Unfortunately, autoantibodies strongly associated with the diagnosis of chronic inflammatory demyelinating polyneuropathies and with demyelinating forms of GBS are still lacking.Identification of autoantibodies that map onto a specific clinical phenotype not only allows for improved classification, but also provides better understanding of the pathophysiology of inflammatory neuropathies and the potential for therapeutic interventions.

摘要

在免疫介导的神经疾病中,已发现多种自身抗体,这些抗体靶向存在于周围神经中富含的糖脂和糖蛋白所携带的聚糖。尽管在敏感性和特异性方面存在一些局限性,且缺乏标准化检测方法和质量保证方案,但它们作为诊断生物标志物的应用非常广泛。虽然已应用多种方法进行检测,但酶联免疫吸附测定(ELISA),无论是以商业试剂盒形式还是内部检测形式,仍然是最广泛可用且便捷的检测方法。一些抗体具有特别显著且广受认可的临床意义。因此,在IgM副蛋白相关神经病中发现的抗髓鞘相关糖蛋白(MAG)IgM抗体定义了一种相对一致的临床和预后表型。抗神经节苷脂GM1和GD1a的IgG抗体与格林-巴利综合征的运动轴索性变异型密切相关,而抗GQ1b抗体与米勒-费希尔综合征相关。在其他慢性神经病中,包括GD1b和GD3在内的双唾液酸化神经节苷脂抗体在共济失调性神经病中被检测到,通常与IgM副蛋白相关,而抗GM1和复合GM1:GalC抗体在多灶性运动神经病中经常被发现。不幸的是,仍缺乏与慢性炎症性脱髓鞘性多发性神经病的诊断以及格林-巴利综合征脱髓鞘形式密切相关的自身抗体。鉴定与特定临床表型相关的自身抗体不仅有助于改进分类,还能更好地理解炎性神经病的病理生理学以及治疗干预的潜力。

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