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血浆置换治疗重症肌无力危象的可预测性恢复:36例病例及当前治疗方法综述

Predictable recovery from myasthenia gravis crisis with plasma exchange: thirty-six cases and review of current management.

作者信息

Mahalati K, Dawson R B, Collins J O, Mayer R F

机构信息

Department of Pathology, The University of Maryland School of Medicine, Baltimore, USA.

出版信息

J Clin Apher. 1999;14(1):1-8. doi: 10.1002/(sici)1098-1101(1999)14:1<1::aid-jca1>3.0.co;2-u.

Abstract

Adult, acquired, idiopathic, autoimmune myasthenia gravis has a well-characterized IgG anti-acetylcholine striated-muscle receptor antibody. Removal by plasma exchange is effective, established therapy to augment anti-cholinesterase and immunosuppressive therapy and is the treatment of choice for myasthenia gravis crisis. We report 36 consecutive patients referred and accepted for plasma exchange, 32 of whom were in or entering myasthenia crisis, over a 10 year period. An average of 7.8 (range 1 to 16) plasma exchange procedures were done, with uniform, significant improvement, including extubation of 13 in myasthenic crisis and discharge from hospital in all. We conclude that this is the best treatment for myasthenia gravis crisis in hospital. From recent cases, most, if not all, crises can be prevented by IVIgG or plasma exchange as out-patients with use of corticosteroid and or azathioprine.

摘要

成人获得性特发性自身免疫性重症肌无力具有特征明确的IgG抗乙酰胆碱横纹肌受体抗体。通过血浆置换清除该抗体是一种有效的既定疗法,可增强抗胆碱酯酶和免疫抑制疗法,是重症肌无力危象的首选治疗方法。我们报告了在10年期间连续转诊并接受血浆置换的36例患者,其中32例处于或即将进入重症肌无力危象。平均进行了7.8次(范围为1至16次)血浆置换程序,均取得了显著改善,包括13例重症肌无力危象患者拔管,所有患者均出院。我们得出结论,这是医院治疗重症肌无力危象的最佳方法。从最近的病例来看,大多数(如果不是全部)危象可以通过静脉注射免疫球蛋白或血浆置换作为门诊患者,并使用皮质类固醇和或硫唑嘌呤来预防。

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