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[免疫球蛋白在神经自身免疫性疾病中的治疗。适应证及作用机制]

[Therapy with immunoglobulins in neurologic autoimmune diseases. Indications and mechanism of action].

作者信息

Gold R, Hartung H P, Toyka K V

机构信息

Neurologische Klinik, Julius-Maximilians-Universität Würzburg.

出版信息

Fortschr Neurol Psychiatr. 1995 Jan;63(1):17-29. doi: 10.1055/s-2007-996599.

DOI:10.1055/s-2007-996599
PMID:7883268
Abstract

Intravenous 7S-immunoglobulins (IVIg) are made of intact human IgG from pooled plasma by using cold alcohol fractionation followed by further purification steps to remove complement-activating material. The half-life of IVIg in vivo is approximately 3 weeks. Favourable effects were reported in patients with myositis and multifocal motor neuropathy who did not respond to established immunosuppressive therapies, and in Guillain-Barre-Syndrome and chronic inflammatory demyelinating polyneuropathy. No definite recommendation can presently be made concerning their use in other neuromuscular disorders and in multiple sclerosis where the results of ongoing and future controlled studies have to be awaited. Some of the possible mechanisms of immunoglobulin efficacy have been delineated in animal models and tissue culture: anti-idiotypic suppression, down-regulation of B- and T-cell activation, blockade of Fc receptors on phagocytic cells, neutralisation of superantigen and complement mediated effects, down-regulation of cytokine production and neutralisation of cytokines. Few side effects have been reported to date. However, treatment is expensive when compared with established immunosuppressive therapies. The main risk consists of the transmission of infectious agents that can only be excluded if the manufacturing process is optimal. A broad and uncontrolled use of immunoglobulins in the treatment of neurologic disorders is discouraged.

摘要

静脉注射7S免疫球蛋白(IVIg)由混合血浆中的完整人IgG制成,采用冷乙醇分级分离法,随后进行进一步纯化步骤以去除补体激活物质。IVIg在体内的半衰期约为3周。据报道,在对既定免疫抑制疗法无反应的肌炎和多灶性运动神经病患者,以及格林-巴利综合征和慢性炎症性脱髓鞘性多发性神经病患者中,IVIg有良好效果。目前对于其在其他神经肌肉疾病和多发性硬化症中的应用无法给出明确建议,必须等待正在进行和未来的对照研究结果。免疫球蛋白疗效的一些可能机制已在动物模型和组织培养中得到阐明:抗独特型抑制、B细胞和T细胞激活的下调、吞噬细胞上Fc受体的阻断、超抗原中和及补体介导的效应、细胞因子产生的下调和细胞因子的中和。迄今为止,报道的副作用很少。然而,与既定的免疫抑制疗法相比,治疗费用昂贵。主要风险在于感染因子的传播,只有在生产过程最佳时才能排除这种风险。不鼓励在治疗神经系统疾病时广泛且无控制地使用免疫球蛋白。

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[The use of intravenous immunglobulin in the treatment of autoimmune neuromuscular diseases].静脉注射免疫球蛋白在自身免疫性神经肌肉疾病治疗中的应用
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