Department of Neurology, University of Würzburg, Josef-Schneider-Str. 11, D-97080 Würzburg, Germany.
Trends Pharmacol Sci. 2013 Aug;34(8):445-57. doi: 10.1016/j.tips.2013.05.009. Epub 2013 Jun 21.
Demyelinating diseases with presumed autoimmune pathogenesis are characterised by direct or indirect immune-mediated damage to myelin sheaths, which normally surround nerve fibres to ensure proper electrical nerve conduction. Parenteral administration of polyclonal IgG purified from multi-donor human plasma pools may beneficially modulate these misguided immune reactions via several mechanisms that are outlined in this review. Convincing therapeutic evidence from controlled trials now exists for certain disorders of the peripheral nervous system, including Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, and multifocal motor neuropathy. In addition, there is evidence for potential therapeutic benefits of IgG in patients with chronic inflammatory demyelinating diseases of the central nervous system, including multiple sclerosis and neuromyelitis optica. This review introduces these disorders, briefly summarises the established treatment options, and discusses therapeutic evidence for the use of polyclonal immunoglobulins with a particular emphasis on recent clinical trials and meta-analyses.
具有推测自身免疫发病机制的脱髓鞘疾病的特征是直接或间接的免疫介导的髓鞘损伤,髓鞘通常围绕神经纤维,以确保适当的电神经传导。从多供体人血浆池中纯化的多克隆 IgG 通过在本综述中概述的几种机制可有益地调节这些误导的免疫反应。现在,已经有令人信服的对照试验治疗证据表明,某些周围神经系统疾病,包括格林-巴利综合征、慢性炎症性脱髓鞘性多发性神经病和多灶性运动神经病具有治疗作用。此外,在包括多发性硬化症和视神经脊髓炎在内的中枢神经系统慢性炎症性脱髓鞘疾病患者中,IgG 也有潜在治疗获益的证据。本综述介绍了这些疾病,简要总结了既定的治疗选择,并讨论了多克隆免疫球蛋白的治疗证据,特别强调了最近的临床试验和荟萃分析。