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胸腺瘤相关性重症肌无力的临床特征和结局_Eur J Neurol

已有 238 次阅读2021-4-14 21:03 |个人分类:TET学习|系统分类:医学科学

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.

Álvarez-Velasco R, Gutiérrez-Gutiérrez G, Trujillo JC, Martínez E, Segovia S, Arribas-Velasco M, Fernández G, Paradas C, Vélez-Gómez B, Casasnovas C, Nedkova V, Guerrero-Sola A, Ramos-Fransi A, Martínez-Piñeiro A, Pardo J, Sevilla T, Gómez-Caravaca MT, López de Munain A, Jericó I, Pelayo-Negro AL, Martín MA, Morgado Y, Mendoza MD, Pérez-Pérez H, Rojas-García R, Turon-Sans J, Querol L, Gallardo E, Illa I, Cortés-Vicente E. Clinical characteristics and outcomes of thymoma-associated myasthenia gravis. Eur J Neurol. 2021 Mar 15. doi: 10.1111/ene.14820. Epub ahead of print. PMID: 33721382.

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