The impact of thymectomy in subgroups of Myasthenia gravis patients: a single center longitudinal observation.

Hruy Menghesha, Michael Schroeter, Christopher Nelke, Tobias Ruck, Georg Schlachtenberger, Clara Welskop, Amina Camo, Matthias Heldwein, Gerardus Bennink, Thorsten Wahlers, Servet Bölükbas, Fabian Doerr, Khosro Hekmat
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引用次数: 1

Abstract

Background: Myasthenia gravis (MG) is a rare neuromuscular disorder. Symptoms can range from ptosis only to life threatening myasthenic crisis. Thymectomy is recommended for anti-acetylcholine receptor-antibody positive patients with early-onset MG. Here, we investigated prognostic factors shaping therapeutic outcomes of thymectomy to improve patient stratification.

Methods: We retrospectively collected single-center data from a specialized center for MG from all consecutive adult patients that underwent thymectomy from 01/2012 to 12/2020. We selected patients with thymoma-associated and non-thymomatous MG for further investigations. We analyzed the patient collective regarding perioperative parameters in relation to the surgical approach. Furthermore, we investigated the dynamics of the anti-acetylcholine receptor-antibody titers and concurrent immunosuppressive therapies, as well as the therapeutic outcomes in dependence of clinical classifications.

Results: Of 137 patients 94 were included for further analysis. We used a minimally invasive approach in 73 patients, whereas 21 patients underwent sternotomy. A total of 45 patients were classified as early-onset MG (EOMG), 28 as late-onset MG (LOMG) and 21 as thymoma-associated MG (TAMG). The groups differed in terms of age at diagnosis (EOMG: 31.1 ± 12.2 years; LOMG: 59.8 ± 13.7 years; TAMG: 58.6 ± 16.7 years; p < 0.001). Patients with EOMG and TAMG were more often female than patients in the LOMG group (EOMG: 75.6%; LOMG: 42.9%; TAMG: 61.9%; p = 0.018). There were no significant differences in outcome scores (quantitative MG; MG activities of daily living; MG Quality of Live) with a median follow-up of 46 months. However, Complete Stable Remission was achieved significantly more frequently in the EOMG group than in the other two groups (p = 0.031). At the same time, symptoms seem to improve similarly in all three groups (p = 0.25).

Conclusion: Our study confirms the benefit of thymectomy in the therapy of MG. Both, the concentration of acetylcholine receptor antibodies and the necessary dosage of cortisone therapy show a continuous regression after thymectomy in the overall cohort. Beyond EOMG, groups of LOMG and thymomatous MG responded to thymectomy as well, but therapy success was less pronounced and delayed compared to the EOMG subgroup. Thymectomy is a mainstay of MG therapy to be considered in all subgroups of MG patients investigated.

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胸腺切除术对重症肌无力亚组患者的影响:单中心纵向观察。
背景:重症肌无力是一种罕见的神经肌肉疾病。症状可以从仅上睑下垂到危及生命的肌无力危机。抗乙酰胆碱受体抗体阳性的早发性MG患者推荐胸腺切除术。在这里,我们研究了影响胸腺切除术治疗结果的预后因素,以改善患者分层。方法:我们回顾性收集2012年1月至2020年12月期间接受胸腺切除术的所有连续成人MG专业中心的单中心数据。我们选择胸腺瘤相关和非胸腺瘤性MG患者进行进一步研究。我们分析了与手术入路相关的围手术期参数。此外,我们还研究了抗乙酰胆碱受体抗体滴度和同步免疫抑制治疗的动态,以及临床分类依赖性的治疗结果。结果:137例患者中94例纳入进一步分析。我们对73例患者采用微创入路,而21例患者采用胸骨切开术。45例为早发性MG (EOMG), 28例为晚发性MG (LOMG), 21例为胸腺瘤相关MG (tam)。两组在诊断年龄方面存在差异(EOMG: 31.1±12.2岁;寿命:59.8±13.7岁;寿命:58.6±16.7岁;结论:我们的研究证实了胸腺切除术治疗MG的益处。在整个队列中,乙酰胆碱受体抗体的浓度和可的松治疗的必要剂量均显示胸腺切除术后持续下降。除EOMG外,LOMG组和胸腺瘤性MG组也对胸腺切除术有反应,但与EOMG亚组相比,治疗成功不那么明显和延迟。胸腺切除术是所有MG患者亚组中MG治疗的主要方法。
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