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实践指南:胸腺瘤切除术治疗重症肌无力(实践参数更新):美国神经病学学会指南制定、传播和实施小组委员会的报告。

Practice advisory: Thymectomy for myasthenia gravis (practice parameter update): Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

机构信息

From the Department of Neurology (G.S.G., R.B.), University of Kansas Medical Center, Kansas City; and Department of Neurology (P.N.), Beth Israel Deaconess Medical Center/Harvard Medical School, Boston, MA.

出版信息

Neurology. 2020 Apr 21;94(16):705-709. doi: 10.1212/WNL.0000000000009294. Epub 2020 Mar 25.

Abstract

OBJECTIVE

To review updated evidence regarding the effectiveness of thymectomy for treating patients with myasthenia gravis (MG).

METHODS

The practice advisory panel performed a systematic review and developed practice recommendations using methods developed by the American Academy of Neurology.

RESULTS

One Class I study of patients younger than 65 years with nonthymomatous acetylcholine receptor antibody-positive (AChR ab+) generalized MG demonstrated better clinical outcomes in patients treated with oral prednisone and undergoing thymectomy compared with patients treated with prednisone alone, including an increased probability of attaining minimal manifestation status (no symptoms or functional limitations).

CONCLUSION

For patients with nonthymomatous AChR ab+ generalized MG, treatment with thymectomy plus prednisone is probably more effective than treatment with prednisone alone for increasing the chance of attaining minimal manifestation status (risk difference at 36 months, 20%; 95% confidence interval, 1.6%-37%; moderate confidence in the evidence).

RECOMMENDATIONS

Clinicians should discuss thymectomy treatment with patients with AChR ab+ generalized MG (Level B). Clinicians should counsel patients with AChR ab+ generalized MG considering minimally invasive thymectomy techniques that it is uncertain whether the benefit attained by extended transsternal thymectomy will also be attained by minimally invasive approaches (Level B).

摘要

目的

综述胸腺瘤切除术治疗重症肌无力(MG)患者的有效性的最新证据。

方法

实践咨询小组采用美国神经病学学会制定的方法进行系统评价并制定实践建议。

结果

一项针对非胸腺瘤乙酰胆碱受体抗体阳性(AChR ab+)全身型 MG 且年龄小于 65 岁的患者的 I 类研究表明,与单独接受泼尼松治疗的患者相比,接受泼尼松联合胸腺切除术治疗的患者具有更好的临床结局,包括达到最小症状状态(无症状或无功能受限)的可能性增加。

结论

对于非胸腺瘤 AChR ab+全身型 MG 患者,与单独使用泼尼松治疗相比,胸腺切除术联合泼尼松治疗可能更有效地提高达到最小症状状态的几率(36 个月时的风险差异为 20%,95%置信区间为 1.6%-37%;证据可信度为中等)。

建议

对于 AChR ab+全身型 MG 患者,临床医生应与患者讨论胸腺切除术治疗(B 级)。对于考虑采用微创胸腺切除术技术的 AChR ab+全身型 MG 患者,临床医生应告知其,目前尚不确定经胸骨延长的胸腺切除术获得的获益是否也能通过微创方法获得(B 级)。

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