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静脉注射免疫球蛋白治疗获得性重症肌无力。

Intravenous immunoglobulin for the treatment of acquired myasthenia gravis.

作者信息

Howard J F

机构信息

Department of Neurology, University of North Carolina at Chapel Hill, 27599-7025, USA.

出版信息

Neurology. 1998 Dec;51(6 Suppl 5):S30-6. doi: 10.1212/wnl.51.6_suppl_5.s30.

Abstract

Acquired myasthenia gravis (MG) is an autoimmune disorder characterized by exertional fatigue and weakness that is made worse with activity and improved with rest, only to recur with the resumption of activity. The pathology results from an antibody-mediated attack to several different epitopes of the acetylcholine receptor (AChR) complex. The consensus of an expert panel is that intravenous immunoglobulin (IVIg) is effective in reversing myasthenic weakness. Although the mechanism of action is not known, it is likely that there is a downregulation of antibody production. IVIg appears to have a role as an acute treatment intervention in rapidly progressive weakness or as a chronic maintenance therapy when all other treatment modalities have failed. Its response is similar to but slower than the response of plasma exchange (PE), but it offers advantages when therapeutic apheresis is not available or when vascular access is problematic.

摘要

获得性重症肌无力(MG)是一种自身免疫性疾病,其特征为运动性疲劳和肌无力,活动时加重,休息后改善,但恢复活动后又会复发。其病理是由抗体介导的对乙酰胆碱受体(AChR)复合物几个不同表位的攻击所致。一个专家小组的共识是静脉注射免疫球蛋白(IVIg)可有效逆转肌无力。尽管作用机制尚不清楚,但可能是抗体产生下调。IVIg似乎可作为快速进展性肌无力的急性治疗干预措施,或在所有其他治疗方式均无效时作为慢性维持治疗。其反应与血浆置换(PE)相似但较慢,但在无法进行治疗性血液成分单采或血管通路有问题时具有优势。

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