Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy.
Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
Thorac Cancer. 2021 Jan;12(1):106-113. doi: 10.1111/1759-7714.13724. Epub 2020 Nov 3.
Thymoma-associated myasthenia gravis (TAMG) is one of the subtypes of myasthenia gravis with autoantibodies against the acetylcholine receptor (AChR-Ab). We analyzed the clinical features of our cohort of TAMG patients and the changes in AChR-Ab titer before and after thymectomy in order to identify factors predicting thymoma relapses.
We retrospectively assessed: age of MG onset, MG clinical status according to MGFA (Myasthenia Gravis Foundation of America), epoch of thymectomy, post-thymectomy status, oncological features and surgical approach. AChR-Ab dosages were measured both before and after thymectomy. Linear regression models were applied to identify clinical determinants of AChR-Ab titers and the Cox regression model was fitted to estimate the factors associated with the risk of thymoma recurrence.
The study sample included 239 MG patients, 27 of whom experienced one or more recurrences (median follow-up time: 4.8 years). The AChR-Ab titers decreased after first thymectomy (P < 0.001); the decrease was more pronounced in female patients (P = 0.05), in patients diagnosed with MG at an older age (P = 0.003), and in those who had lower MG stage before surgery (P = 0.02) or higher Masaoka-Koga stage (P = 0.005). The risk of relapse was closely linked with the age of the patient, the Masaoka-Koga stage and the surgical approach.
Presurgery levels of AChR-Ab or their change after surgery were not associated with thymoma recurrence. The reduction of AChR-Ab titers after thymectomy confirms an immunological role of thymoma in the pathogenesis of MG.
Significant findings of the study: Young MG patients with an advanced Masaoka staging score of the primary tumor who underwent thymectomy with approaches different from sternotomy and VATS should be monitored for high risk of recurrence.
No other study has ever investigated the changes in AChR-Ab titers before and after thymectomy in a large cohort of TAMG patients. The reduction of AChR-Ab titers after thymectomy suggests an immunological role of thymoma in the pathogenesis of MG.
胸腺瘤相关重症肌无力(TAMG)是一种伴有乙酰胆碱受体(AChR-Ab)自身抗体的重症肌无力亚型。我们分析了 TAMG 患者队列的临床特征以及胸腺切除术前和术后 AChR-Ab 滴度的变化,以确定预测胸腺瘤复发的因素。
我们回顾性评估了:MG 发病年龄、根据美国重症肌无力基金会(MGFA)评估的 MG 临床状态、胸腺切除术时期、胸腺切除术后状态、肿瘤特征和手术方式。在胸腺切除术前和术后均测量了 AChR-Ab 剂量。应用线性回归模型确定 AChR-Ab 滴度的临床决定因素,应用 Cox 回归模型估计与胸腺瘤复发风险相关的因素。
研究样本包括 239 例 MG 患者,其中 27 例患者经历了一次或多次复发(中位随访时间:4.8 年)。首次胸腺切除术后 AChR-Ab 滴度降低(P<0.001);女性患者(P=0.05)、诊断时年龄较大的患者(P=0.003)、术前 MG 分期较低的患者(P=0.02)或 Masaoka-Koga 分期较高的患者(P=0.005)的降低更为显著。复发的风险与患者年龄、Masaoka-Koga 分期和手术方式密切相关。
术前 AChR-Ab 水平或术后变化与胸腺瘤复发无关。胸腺切除术后 AChR-Ab 滴度降低证实了胸腺瘤在 MG 发病机制中的免疫作用。
本研究的重要发现:接受非正中胸骨切开术和 VATS 的不同手术方式的、具有高级别 Masaoka 分期评分的年轻 MG 患者,应密切监测复发的高风险。
尚无其他研究在 TAMG 患者的大队列中调查过胸腺切除术前和术后 AChR-Ab 滴度的变化。胸腺切除术后 AChR-Ab 滴度降低提示胸腺瘤在 MG 发病机制中具有免疫作用。