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胸腺的生理和病理作用及胸腺切除术在重症肌无力中的价值:一项叙述性综述

Physiological and pathological roles of the thymus and value of thymectomy in myasthenia gravis: a narrative review.

作者信息

Waheed Waqar, Bacopulos Agnes, Seyam Muhannad, Kooperkamp Hannah, Moin Maryam, Malik Tariq, Tandan Rup

机构信息

Department of Neurological Sciences, University of Vermont Robert D. Larner, MD College of Medicine and University of Vermont Medical Center, Burlington, VT, USA.

Division of Thoracic and Cardiac Surgery, University of Vermont Robert D. Larner, MD College of Medicine and University of Vermont Medical Center, Burlington, VT, USA.

出版信息

Mediastinum. 2024 May 21;8:31. doi: 10.21037/med-23-43. eCollection 2024.

Abstract

BACKGROUND AND OBJECTIVE

Myasthenia gravis (MG) is a well-elucidated autoimmune disorder affecting the neuromuscular junction. Given the relationship between MG and thymic pathologies, with T cell and antibody-mediated pathogenesis, surgical (i.e., thymectomy) and non-surgical approaches remain a mainstay of management of the disease. This review seeks to outline the involvement of the thymus in the development of lymphocytes leading to MG.

METHODS

Different databases were searched exploring the role of thymectomy in treatment and outcomes in various MG patient subpopulations, including in ocular versus generalized disease, different age groups, and antibody status.

KEY CONTENT AND FINDINGS

Overall, the findings of multiple studies and reviews provide evidence to support the efficacy and long-term success of thymectomy in the management of MG; outcomes have included remission status, symptom severity, and need for adjunctive therapy. However, the heterogeneity in the MG population suggests that there are multiple factors that may confound the results of thymectomy and still need further examination. Separately, other autoimmune diseases develop following thymectomy, and further research is required to elucidate this susceptibility. Finally, our review will discuss the different surgical approaches for thymectomy, including their advantages, limitations, and perioperative complications.

CONCLUSIONS

Overall, in light of the known pathogenesis and association of the thymus with MG, thymectomy remains an extremely effective approach for long-term management and improved clinical outcomes.

摘要

背景与目的

重症肌无力(MG)是一种已被充分阐明的影响神经肌肉接头的自身免疫性疾病。鉴于MG与胸腺病变之间的关系,以及T细胞和抗体介导的发病机制,手术治疗(即胸腺切除术)和非手术治疗方法仍然是该疾病治疗的主要手段。本综述旨在概述胸腺在导致MG的淋巴细胞发育过程中的作用。

方法

检索了不同数据库,探讨胸腺切除术在不同MG患者亚群(包括眼肌型与全身型疾病、不同年龄组以及抗体状态)的治疗及预后中的作用。

关键内容与发现

总体而言,多项研究和综述的结果提供了证据,支持胸腺切除术在MG治疗中的有效性和长期成功率;其结果包括缓解状态、症状严重程度以及辅助治疗的需求。然而,MG患者群体的异质性表明,存在多种因素可能混淆胸腺切除术的结果,仍需进一步研究。另外,胸腺切除术后会发生其他自身免疫性疾病,需要进一步研究以阐明这种易感性。最后,我们的综述将讨论胸腺切除术的不同手术方法,包括其优点、局限性和围手术期并发症。

结论

总体而言,鉴于已知的发病机制以及胸腺与MG的关联,胸腺切除术仍然是长期管理和改善临床结局的极其有效的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2da/11177005/1ad3ba0c18e3/med-08-31-f1.jpg

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