重症肌无力危象前状态管理专家共识(2024)

Expert Consensus on the Management of Impending Myasthenic Crisis(2024)

重症肌无力(MG)是一种自身免疫性神经-肌肉接头疾病,肌无力危象(MC)是MG患者的最严重状态,显著增加患者死亡风险和疾病负担,如能及时识别和干预MG危象前状态,阻止疾病向MC进展,其临床意义重大。然而MG危象前状态的诊断尚无统一标准,对其治疗管理亦缺乏高级别证据。为此,中国MG协作组专家基于新近研究结果和临床实践经验,经过反复讨论,围绕MG危象前状态的早期识别、诊断标准、治疗干预及随访管理等形成共识,以期为临床改善MG危象前状态的管理及预后提供参考。

Myasthenia gravis(MG)is an autoimmune neuromuscular disease,and myasthenic crisis(MC)is the most severe state of MG,significantly increasing the risk of death and disease burden for patients.If the impending myasthenic crisis of MG can be identified and intervened in time,it can prevent the disease from progressing to MC.However,there is no concensus for the diagnosis of impending MC,and there is also a lack of high-level evidence for its treatment and management.Therefore,based on recent research results and clinical practice experience,experts from the Chinese Myasthenia Gravis Collaborative Group(CMGCG),after repeated discussions,formed a consensus on the early identification,diagnostic criteria,therapeutic intervention,and follow-up management of impending MC,in order to provide a reference for improving the management and prognosis of the impending MC in clinical practice.