胸腺瘤相关性重症肌无力的临床特征和结局_Eur J Neurol

胸腺瘤相关性重症肌无力的临床特征和结局。Clinical characteristics and outcomes of thymoma-associated myasthenia gravis

背景与目的：胸腺瘤患者重症肌无力（MG）的预后尚不明确。此外，还不清楚胸腺瘤的复发或无法切除的病变是否会导致MG的预后恶化。

方法：这项多中心研究基于西班牙神经科医生驱动的MG注册中心的数据。所有患者发病年龄均大于18岁，并具有抗乙酰胆碱受体抗体。我们比较了胸腺瘤和非胸腺瘤患者的临床资料。评估复发性或不可切除胸腺瘤患者的预后。

结果：我们纳入了15家医院的964名患者； 148名（15.4％）患有胸腺瘤相关性MG。中位随访时间为4。6年。发作时，与胸腺瘤相关的MG患者较年轻（52.0 vs. 60.4岁，p <0.001），具有更广泛的症状（优势比[OR]：3.02，95％置信区间[CI]：1.95-4.68，p <0.001） ）和更严重的临床形式（根据美国重症肌无力基金会（MGFA）的评分标准（或：1.6，95％CI：1.15-2.21，p = 0.005）。在1年，5年和随访结束时，根据MGFA干预后状态（MGFA-PIS）的疾病严重程度较高。治疗的难治性和死亡率也更高（OR：2.28，95％CI：1.43-3.63，p = 0.001；危险比：2.46，95％CI：1.47-4.14，p = 0.001）。 27例复发患者中有13例的肌无力症状加重，但长期严重程度差异不明显。 15例胸腺瘤患者患有不可切除的胸腺瘤，其MGFA-PIS较差，随访结束时死亡率较高。

结论：胸腺瘤相关性MG患者的重症肌无力症状较重，预后较差。胸腺瘤复发通常与MG的短暂恶化有关，但长期预后与非复发性胸腺瘤没有区别。不可切除胸腺瘤患者倾向于出现严重的MG。

关键词：重症肌无力；预后复发胸腺瘤。

FIGURE 2.Proportion of patients with Myasthenia Gravis Foundation of America postintervention status (MGFA‐PIS) worse than minimal manifestations status after 1 and 5 years of follow‐up.

Background and purpose: Prognosis of myasthenia gravis (MG) in patients with thymoma is not well established. Moreover, it is not clear whether thymoma recurrence or unresectable lesions entail a worse prognosis of MG.

Methods: This multicenter study was based on data from a Spanish neurologist-driven MG registry. All patients were aged >18 years at onset and had anti-acetylcholine receptor antibodies. We compared the clinical data of thymomatous and nonthymomatous patients. Prognosis of patients with recurrent or nonresectable thymomas was assessed.

Results: We included 964 patients from 15 hospitals; 148 (15.4%) had thymoma-associated MG. Median follow-up time was 4.6 years. At onset, thymoma-associated MG patients were younger (52.0 vs. 60.4 years, p < 0.001), had more generalized symptoms (odds ratio [OR]: 3.02, 95% confidence interval [CI]: 1.95-4.68, p < 0.001) and more severe clinical forms according to the Myasthenia Gravis Foundation of America (MGFA) scale (OR: 1.6, 95% CI: 1.15-2.21, p = 0.005). Disease severity based on MGFA postintervention status (MGFA-PIS) was higher in thymomatous patients at 1 year, 5 years, and the end of follow-up. Treatment refractoriness and mortality were also higher (OR: 2.28, 95% CI: 1.43-3.63, p = 0.001; hazard ratio: 2.46, 95% CI: 1.47-4.14, p = 0.001). Myasthenic symptoms worsened in 13 of 27 patients with recurrences, but differences in long-term severity were not significant. Fifteen thymomatous patients had nonresectable thymomas with worse MGFA-PIS and higher mortality at the end of follow-up.

Conclusions: Thymoma-associated MG patients had more severe myasthenic symptoms and worse prognosis. Thymoma recurrence was frequently associated with transient worsening of MG, but long-term prognosis did not differ from nonrecurrent thymoma. Patients with nonresectable thymoma tended to present severe forms of MG.

Keywords: myasthenia gravis; prognosis; recurrence; thymoma.