伴有和不伴有口咽受累的非胸腺瘤性全身型重症肌无力的不同特征。

Different characteristics of nonthymomatous generalized myasthenia gravis with and without oropharyngeal involvement.

作者信息

Yu Lei, Li Jianye, Ma Shan, Jiang Jian, Wang Tianyou, Gamliel Ziv, Jing Yun, Zhang Xiaojun, Krasna Mark J

机构信息

Department of Thoracic Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

出版信息

Ann Thorac Surg. 2007 Nov;84(5):1694-8. doi: 10.1016/j.athoracsur.2007.06.043.

DOI:10.1016/j.athoracsur.2007.06.043

PMID:17954087

Abstract

BACKGROUND

Thymectomy represents a safe and valid approach for patients with myasthenia gravis. However, some factors may influence the efficacy of thymectomy. The objective of this study was to evaluate the clinical characteristics of generalized myasthenia gravis with oropharyngeal involvement and compare the postoperative outcome of generalized myasthenia gravis without and with oropharyngeal involvement.

METHODS

From 1991 to 2002, there were 202 patients with nonthymomatous generalized myasthenia gravis (GMG), who underwent thymectomy by the transsternal approach or thoracoscopy. According to the clinical classification of the Myasthenia Gravis Foundation of America (MGFA), these patients have been subdivided into two groups: GMG without oropharyngeal involvement and GMG with oropharyngeal involvement. Complete stable remission, as defined by the MGFA Medical Task Force, was the primary endpoint for efficacy.

RESULTS

In all, 182 patients were followed up for at least 5 years. There were 135 cases of GMG without oropharyngeal involvement and 47 cases of GMG with oropharyngeal involvement. There were significant differences in postoperative pneumonia, ventilatory support time, and myasthenic crisis between patients with GMG with and without oropharyngeal involvement (p values were 0.040, 0.021, and 0.007, respectively). At 5 years of follow-up, the cumulative probability of reaching complete stable remission in GMG without oropharyngeal involvement was 18.5% at the end of the first year, and rose steadily in subsequent years (26.7%, 37.0%, 39.1%, and 40% at 2, 3, 4, and 5 years, respectively). In GMG with oropharyngeal involvement, it was 6.4%, 14.9%, 23.4%, 29.9%, and 31.9%, respectively, in the continual follow-up years. The first 3 years were associated with a significantly greater probability of achieving complete stable remission (p = 0.047 for the first year, p = 0.025 for the second one, and p = 0.048 for the third one). The later 2 years had no significant difference on complete stable remission.

CONCLUSIONS

Laryngeal myasthenia gravis is more severe and the prognosis after thymectomy is not as optimistic as for patients without oropharyngeal involvement.

摘要

背景

胸腺切除术是治疗重症肌无力患者的一种安全有效的方法。然而，一些因素可能会影响胸腺切除术的疗效。本研究的目的是评估伴有口咽受累的全身型重症肌无力的临床特征，并比较无口咽受累和有口咽受累的全身型重症肌无力患者的术后结局。

方法

1991年至2002年，202例非胸腺瘤性全身型重症肌无力（GMG）患者接受了经胸骨或胸腔镜胸腺切除术。根据美国重症肌无力基金会（MGFA）的临床分类，这些患者被分为两组：无口咽受累的GMG和有口咽受累的GMG。MGFA医学工作组定义的完全稳定缓解是疗效的主要终点。

结果

总共182例患者接受了至少5年的随访。其中无口咽受累的GMG患者135例，有口咽受累的GMG患者47例。有口咽受累和无口咽受累的GMG患者在术后肺炎、通气支持时间和肌无力危象方面存在显著差异（p值分别为0.040、0.021和0.007）。在5年的随访中，无口咽受累的GMG患者在第一年结束时达到完全稳定缓解的累积概率为18.5%，并在随后几年稳步上升（在第2、3、4和5年分别为26.7%、37.0%、39.1%和40%）。在有口咽受累的GMG患者中，在连续随访年份中分别为6.4%、14.9%、23.4%、29.9%和31.9%。前3年实现完全稳定缓解的概率显著更高（第一年p = 0.047，第二年p = 0.025，第三年p = 0.048）。后2年在完全稳定缓解方面无显著差异。