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目的研究重症肌无力(MG)患者血清连接素抗体(Titin-ab)和 Ryanodine 受体抗体(RyR-ab)的水平,并探讨胸腺瘤内 Titin、RyR 表位的表达。方法应用 ELISA 法检测62例 MG 患者血清中 Titin-ab、RyR-ab 水平,以45例非 MG 的其他神经系统疾病患者和50名健康志愿者为对照组,采用免疫组织化学技术,对19例合并胸腺异常者[9例 MG 合并胸腺瘤(MGT)、6例 MG 合并胸腺增生(MGH)、2例 MG 合并胸腺萎缩(MGA)以及2例非 MG 胸腺癌(NMGTC)]的冰冻胸腺组织进行Titin、RyR 染色。结果 ELISA 法显示,MG 患者 Titin-ab 总阳性率为35.5%(22/62),其中以 MGT 组阳性率最高(82.3%,14/17);而 RyR-ab 阳性率为24.2%(15/62),也以 MGT 组阳性率最高(76.5%,13/17)。9例 MGT 的肿瘤上皮细胞中有7例存在 Titin 表位的膜表达和胞质内表达,有6例存在 RyR表位的膜表达;而 MGH 组、MGA 组、NMGTC 组和对照组均无阳性表达。结论 Titin-ab 和 RyR-ab 主要见于 MGT 患者。MGT 肿瘤上皮细胞存在 Titin、RyR 表位的表达。MGT 患者体内 Titin-ab 和 RyR-ab 很可能是胸腺瘤内微环境的改变激活 Titin、RyR 特异性 T 细胞,致敏 Titin、RyR 表位使其发生自身免疫反应的结果。
Objective To investigate the levels of Titin-ab and RyR-ab in patients with myasthenia gravis (MG) and to explore the expression of Titin and RyR in thymoma. Methods The serum levels of Titin-ab and RyR-ab in 62 patients with MG were determined by ELISA. The other 45 patients with non-MG neurological diseases and 50 healthy volunteers were used as control group. Immunohistochemistry was performed on 19 patients Frozen thymus with thymus abnormalities (9 MG thymoma (MGT), 6 MG thymus (MGH), 2 MG thymus atrophy (MGA) and 2 non-MG thymoma (NMGTC)] Titin, RyR staining. Results The total positive rate of Titin-ab in MG patients was 35.5% (22/62). The positive rate of MGT group was the highest (82.3%, 14/17), while the positive rate of RyR-ab was 24.2% (15 / 62), and the highest positive rate was MGT (76.5%, 13/17). Nine of the nine MGT tumor-bearing epithelial cells showed the expression of Titin and the intracytoplasmic expression, while the expression of RyR was found in six of the MGT specimens. However, MGH, MGA, NMGTC and control groups showed no positive staining expression. Conclusion Titin-ab and RyR-ab are mainly found in patients with MGT. MGT tumor epithelial cells exist Titin, RyR epitope expression. Titin-ab and RyR-ab in MGT patients are likely to be the result of the autoimmune reaction of Titin, RyR-specific T cells and Titin, RyR epitopes in the thymoma.