Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, (L-5), 2-2 Yamada-oka, Suita, Osaka, Japan.
Surg Today. 2010;40(2):102-7. doi: 10.1007/s00595-009-4134-2. Epub 2010 Jan 28.
Myasthenia gravis (MG) is an autoimmune disease mediated by autoantibodies to the striated muscle tissue. It is often treated by thymectomy. We review recent studies to investigate the biological implications of thymectomy. In anti-acetylcholine receptor antibody (anti-AchR Ab)-positive patients without a thymoma, abnormal germinal center formation in the thymus seems to play an essential role in the pathogenesis of MG. Specific differentiation of B cells producing anti-AchR Ab takes place uniquely in the thymus, and thymectomy is thought to assist in terminating the provision of high-affinity anti-AchR antibody-producing cells to peripheral organs. Thymectomy is not indicated for anti-AchR Ab-negative MG patients who are antimuscle specific kinase antibody (anti-MuSK Ab)-positive, although some anti-MuSK Ab-negative patients may benefit from the procedure. A thymoma can be considered as an acquired thymus with insufficient function of negative selection. The resection of a thymoma is thought to terminate the production of self-reactive T cells. Thus, the biological implications of thymectomy for MG have been partially revealed. Nevertheless, additional studies are needed to elucidate the ontogeny of T cells that recognize AchR and the mechanism of the activation of anti-AchR antibodies producing B cells.
重症肌无力(MG)是一种由抗横纹肌组织自身抗体介导的自身免疫性疾病。它通常通过胸腺切除术进行治疗。我们回顾了最近的研究,以探讨胸腺切除术的生物学意义。在没有胸腺瘤的抗乙酰胆碱受体抗体(anti-AchR Ab)阳性患者中,胸腺中异常生发中心的形成似乎在 MG 的发病机制中起着至关重要的作用。产生抗-AchR Ab 的 B 细胞的特异性分化仅在胸腺中发生,并且胸腺切除术被认为有助于终止高亲和力抗-AchR 抗体产生细胞向外周器官的供应。抗-AchR Ab 阴性 MG 患者抗肌肉特异性激酶抗体(anti-MuSK Ab)阳性时不需要进行胸腺切除术,尽管一些 anti-MuSK Ab 阴性患者可能受益于该手术。胸腺瘤可被视为具有不完全负选择功能的获得性胸腺。胸腺瘤的切除被认为终止了自身反应性 T 细胞的产生。因此,胸腺切除术对 MG 的生物学意义已经部分揭示。然而,需要进一步的研究来阐明识别 AchR 的 T 细胞的发生以及产生抗-AchR 抗体的 B 细胞的激活机制。