Stepanova S B, Karpova M I, Vasilenko A F, Domashenko M A
South Ural State Medical University, Chelyabinsk, Russia.
Central Clinical Medical and Sanitary Unit, Magnitogorsk, Russia.
Zh Nevrol Psikhiatr Im S S Korsakova. 2024;124(7. Vyp. 2):85-90. doi: 10.17116/jnevro202412407285.
Myasthenia gravis (MG) is a chronic autoimmune disease mediated by autoreactive T- and B-cells and manifested by progressive pathological muscle weakness and fatigue. Traditional immunomodulatory treatment does not always lead to the clinical picture significant improvement, despite adequate dosage and duration of use. Refractory myasthenia gravis requires new therapeutic approaches development and implementation. The range of target innovative agents in refractory MG includes monoclonal antibodies, which act directly on individual components of the complement system. Based on the results of randomized controlled trials, data on the effectiveness and safety of eculizumab, which inhibits the C5 component of the complement system, is presented. We present our own clinical experience of using eculizumab in a young woman with refractory generalized AChR-positive MG with a rapid decrease in the severity of symptoms to a minimum level and restoration of ability to work, and the absence of adverse events during therapy.
重症肌无力（MG）是一种由自身反应性T细胞和B细胞介导的慢性自身免疫性疾病，表现为进行性病理性肌肉无力和疲劳。尽管传统免疫调节治疗的使用剂量和疗程足够，但并不总能使临床症状得到显著改善。难治性重症肌无力需要开发和实施新的治疗方法。难治性MG的一系列靶向创新药物包括直接作用于补体系统各个成分的单克隆抗体。基于随机对照试验的结果，本文介绍了抑制补体系统C5成分的依库珠单抗的有效性和安全性数据。我们介绍了在一名年轻女性难治性全身性乙酰胆碱受体阳性MG患者中使用依库珠单抗的临床经验，该患者症状严重程度迅速降至最低水平，工作能力恢复，且治疗期间未出现不良事件。
