Jaiswal Shreya, Shrivastava Tripti
Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Physiology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Cureus. 2024 Apr 26;16(4):e59104. doi: 10.7759/cureus.59104. eCollection 2024 Apr.
Myasthenia gravis (MG) is an autoimmune illness characterized by autoantibodies against the acetylcholine receptor (AChR), muscle-specific tyrosine kinase (MuSK), and an increasing number of extra postsynaptic proteins. Pathogenic autoantibodies reduce the number of functional AChRs in the neuromuscular junction's (NMJ) muscle end plate. The cause of the autoimmune response is unknown, but thymic abnormalities and immune regulatory deficiencies are significant. The disease's incidence is likely influenced by genetic predisposition, with sex hormones and exercise playing a role. MG can affect any age, race, or gender and can be caused by any stressor, with infections being the most frequent cause. Treatment focuses on airway support and the triggering incident. MG is a rare autoimmune disease causing fatigue-inducing weakness in the axial, respiratory, leg, and bulbar muscles. Initially affecting the eyes, most MG patients experience at least one worsening symptom during their illness. The disease is mainly caused by antibodies against the AChR, dependence on the immune system within cells, and engagement of the complement system. The complement system plays a significant role in MG, and complement inhibition can both prevent the onset and slow its development. Ocular MG affects around 15% of people, with most patients having blocking antibodies against the cholinergic receptor. There may be correlations between thymoma and other autoimmune conditions, especially thyroid illness. Treatment and management for MG involve removing autoantibodies from circulation or blocking effector mechanisms using techniques such as complement inhibition, plasmapheresis, and B-cell elimination.
重症肌无力(MG)是一种自身免疫性疾病,其特征是存在针对乙酰胆碱受体(AChR)、肌肉特异性酪氨酸激酶(MuSK)以及越来越多突触后膜外蛋白的自身抗体。致病性自身抗体减少了神经肌肉接头(NMJ)肌终板中功能性AChR的数量。自身免疫反应的原因尚不清楚,但胸腺异常和免疫调节缺陷很重要。该疾病的发病率可能受遗传易感性影响,性激素和运动也起一定作用。MG可影响任何年龄、种族或性别,任何应激源都可能引发该病,其中感染是最常见的原因。治疗重点在于气道支持和引发事件。MG是一种罕见的自身免疫性疾病,可导致躯干、呼吸、腿部和延髓肌肉出现引起疲劳的肌无力。最初影响眼部,大多数MG患者在患病期间至少会出现一种症状加重的情况。该疾病主要由针对AChR的抗体、细胞内对免疫系统的依赖以及补体系统的参与引起。补体系统在MG中起重要作用,抑制补体可预防疾病发作并减缓其发展。眼肌型MG影响约15%的人,大多数患者有针对胆碱能受体的阻断抗体。胸腺瘤与其他自身免疫性疾病之间可能存在关联,尤其是甲状腺疾病。MG的治疗和管理包括通过补体抑制、血浆置换和B细胞清除等技术从循环中清除自身抗体或阻断效应机制。