Clinical Outcomes of Myasthenia Gravis with Thymoma and Thymic Hyperplasia Undergoing Extended Transsternal Thymectomy: A Single-Center Experience

S. Nazarbaghi, M. R. Amiri-Nikpour, R. Mahmodlou, Nasim Arjmand, Yousef Rezaei
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引用次数: 14

Abstract

Background: Despite the widespread use of thymectomy in myasthenia gravis (MG) patients, it has remained controversial as to whether this procedure is of a similar efficacy and clinical outcome among MG patients with thymoma and thymic hyperplasia. Aim: We sought to determine the long-term clinical outcomes of MG patients who received extended transsternal thymectomy associated with pyridostigmine and prednisolone postoperatively. Materials and Methods: In a retrospective study from January 1999 to December 2013, MG patients who underwent thymectomy were followed up. Out of 41 MG patients admitted in our center, 25 patients had undergone thymectomy adjunctive to pyridostigmine and prednisolone therapy postoperatively. The primary endpoints included improvement in individual diplopia, ptosis, dysphagia, dysarthria, dyspnea, and limb weakness. In addition, according to the MG Foundation of America (MGFA) criteria, response to therapy was defined as complete stable remission (CSR), pharmacologic remission (PR), and minimal manifestation (MM) as secondary endpoints. Results: Majority of the patients were male (60%) and the mean age of the patients was 32.2 ± 13.9 years. Fifteen (60%) and 10 patients (40%) had thymoma and thymic hyperplasia, respectively. All the patients were followed up during a mean period of of 86.9 ± 50.3 months (minimum 10 months and maximum 168 months). The rates of CSR, PR, and MM were comparable between the thymoma and thymic hyperplasia groups (P = 0.584). Based on the Kaplan Meier analysis, the probabilities of CSR, PR, and MM were not significantly different between patients with thymoma and thymic hyperplasia. Conclusion: The extended transsternal thymectomy, along with the postoperative regimen of pyridostigmine and prednisolone was associated with a high rate of clinical improvement among MG patients with thymoma or thymic hyperplasia.
重症肌无力合并胸腺瘤和胸腺增生行扩展胸腺经胸骨切除术的临床结果:单中心经验
背景:尽管在重症肌无力(MG)患者中广泛应用胸腺切除术,但对于该手术在合并胸腺瘤和胸腺增生的重症肌无力患者中是否具有相似的疗效和临床结果仍然存在争议。目的:我们试图确定MG患者术后接受延长经胸骨胸腺切除术联合吡哆斯的明和强的松龙的长期临床结果。材料与方法:对1999年1月至2013年12月行胸腺切除术的MG患者进行回顾性随访。在我们中心收治的41例MG患者中,25例患者术后行胸腺切除术,并辅以吡哆斯的明和强的松龙治疗。主要终点包括个体复视、上睑下垂、吞咽困难、构音障碍、呼吸困难和肢体无力的改善。此外,根据美国MG基金会(MGFA)的标准,对治疗的反应被定义为完全稳定缓解(CSR),药理学缓解(PR)和最小表现(MM)作为次要终点。结果:患者以男性居多(60%),平均年龄32.2±13.9岁。胸腺瘤15例(60%),胸腺增生10例(40%)。所有患者的平均随访时间为86.9±50.3个月(最短10个月,最长168个月)。胸腺瘤组和胸腺增生组的CSR、PR和MM率具有可比性(P = 0.584)。根据Kaplan Meier分析,胸腺瘤和胸腺增生患者发生CSR、PR和MM的概率无显著差异。结论:胸腺瘤或胸腺增生的MG患者,经胸骨胸腺切除术及术后给予吡地斯的明和强的松龙治疗可提高临床改善率。
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