胸腺在土耳其人群重症肌无力预后中的作用。
Role of thymus on prognosis of myasthenia gravis in Turkish population.
作者信息
Tireli Hulya, Yuksel Gulbun, Okay Tamer, Tutkavul Kemal
机构信息
Department of Neurology, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey.
Department of Thorax Surgery, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
出版信息
North Clin Istanb. 2020 Aug 10;7(5):452-459. doi: 10.14744/nci.2020.51333. eCollection 2020.
OBJECTIVE
Myasthenia gravis (MG) is an autoimmune disease that may cause a disorder in transmission at the neuromuscular junction. Antibodies directed against acetylcholine receptors are responsible. The thymus is the place that that production of these antibodies mainly occurs. The thymus gland abnormalities and abnormal production of these antibodies are associated with MG. Consequently, thymectomy is a common treatment for MG. The nature of the disease makes it difficult to plan prospective, controlled trials; therefore, there is no current consensus among clinicians on a single algorithm of treatment, and the approach is frequently based on the observations and experiences of experts. The contributions to the literature largely consist of retrospective studies examining an approach to treatment and the effects of thymectomy on prognosis. In this retrospective study, evaluation of Turkish patients with myasthenia gravis was carried out for the importance of thymectomy and effects on prognosis.
METHODS
In this study, 93 patients with myasthenia gravis whose followed up at Neuromuscular outpatient clinic between 1998-2018 were evaluated retrospectively. Type of disease, antibody status, treatment, thymectomy, thymus pathology and prognosis were assessed.
RESULTS
Thymectomy had been a positive effect on the prognosis of the disease independent of the duration of disease and thymic pathology. The best results had been obtained with early thymectomy with short disease duration, younger age and patients with thymic hyperplasia. Success of therapy was limited with thymoma. With advanced age need for thymectomy was decreased.
CONCLUSION
In the present study, evaluation of 93 patients with myasthenia gravis was done retrospectively and it was concluded that thymectomy had a positive effect on prognosis, especially in young patients when performed as early as possible. The most successful results were obtained in cases with thymic hyperplasia.
目的
重症肌无力（MG）是一种自身免疫性疾病，可导致神经肌肉接头处传递障碍。针对乙酰胆碱受体的抗体是其病因。胸腺是这些抗体主要产生的部位。胸腺异常及这些抗体的异常产生与重症肌无力相关。因此，胸腺切除术是重症肌无力的常见治疗方法。该疾病的性质使得前瞻性、对照试验难以开展；因此，临床医生目前对于单一治疗方案尚无共识，治疗方法通常基于专家的观察和经验。文献中的研究大多为回顾性研究，探讨治疗方法及胸腺切除术对预后的影响。在这项回顾性研究中，对土耳其重症肌无力患者进行评估，以探讨胸腺切除术的重要性及其对预后的影响。
方法
本研究回顾性评估了1998年至2018年在神经肌肉门诊随访的93例重症肌无力患者。评估疾病类型、抗体状态、治疗、胸腺切除术、胸腺病理及预后。
结果
胸腺切除术对疾病预后有积极影响，与疾病持续时间和胸腺病理无关。疾病持续时间短、年龄较小且胸腺增生的患者早期行胸腺切除术效果最佳。胸腺瘤患者的治疗效果有限。随着年龄增长，胸腺切除术的需求降低。
结论
在本研究中，对93例重症肌无力患者进行了回顾性评估，得出结论：胸腺切除术对预后有积极影响，尤其是对年轻患者尽早进行时。胸腺增生患者取得了最成功的结果。
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