重症肌無力 (myasthenia gravis) 是一種神經肌肉傳遞障礙的自體免疫疾病，其致病機轉為人體免疫系統異常產生自體抗體攻擊神經肌肉突觸間的乙醯膽鹼受體(acetylcholine receptor)，造成受體減少而導致傳導障礙與不同程度的肌肉無力，最常影響臉部等肌肉而造成眼瞼下垂、吞嚥困難等症狀，嚴重時甚至可能影響呼吸肌而導致呼吸困難。臨床上重症肌無力的藥物治療可以分為症狀控制及免疫療法。前者主要使用乙醯膽鹼酯酶抑制劑 (acetylcholinesterase inhibitor) 以減緩肌肉無力症狀；後者則使用類固醇或非類固醇免疫抑制劑來減少自體抗體的產生。近年來越來越多針對免疫系統的標靶藥物被開發出來並用於重症肌無力的治療，依機轉區分為三大類：新生兒Fc 受體抑制劑 (neonatal fragment crystallizable receptor inhibitor)、補體抑制劑(complement inhibitors) 及B 細胞清除藥物 (B-cell depletion therapy)，在臨床試驗顯示大部分藥物具有良好的耐受性，並且可顯著改善重症肌無力症狀，為重症肌無力病人提供了具有潛力的治療新選擇。

 

 

Myasthenia gravis is an autoimmune disorder resulting from autoantibodies that attack the acetylcholine receptors at the neuromuscular junction of skeletal muscles, leading to the decrease in AChR and abnormal transmission. In most patients, facial muscles are affected first, leading to symptoms such as eyelid ptosis and dysphagia. In severe cases, it affects respiratory muscles and leads to difficulty in breathing. Clinical drug treatment of myasthenia gravis including using cholinesterase inhibitors to alleviate the symptoms of muscle weakness. Glucocorticoids or non-corticosteroid immunosuppressive agents are used to reduce the production of autoantibodies. Recently, many targeted therapy drugs that target the immune system have been developed and used for the treatment of myasthenia gravis. It is divided into three categories according to the mechanism: neonatal fragment crystallizable receptor inhibitor, complement inhibitor and B cell depleting agents. Clinical trials have shown that most of targeted therapy drugs is well tolerated and can significantly alleviate the symptoms of myasthenia gravis, which providing a new potential treatment option for patients with myasthenia gravis.

 

 

Submitted for publication: 2024.3.18; Accepted for publication: 2024.6.6