Huang Yangyu, Tan Ying, Shi Jiayu, Li Ke, Yan Jingwen, Guan Yuzhou
Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
Front Neurol. 2021 Nov 18;12:767961. doi: 10.3389/fneur.2021.767961. eCollection 2021.
Life-threatening myasthenic crisis (MC) occurs in 10-20% of the patients with myasthenia gravis (MG). It is important to identify the predictors of progression to MC and prognosis in the patients with MG with acute exacerbations. This study aimed to explore the predictors of progression to MC in the patients with MG with acute onset of dyspnea and their short-term and long-term prognosis. This study is a retrospective cohort study. We collected and analyzed data on all the patients with MG with acute dyspnea over a 10-year period in a single center using the univariate and multivariate analysis. Eighty-six patients with MG were included. In their first acute dyspnea episodes, 36 (41.9%) episodes eventually progressed to MC. A multivariate analysis showed that the early-onset MG (adjusted : 3.079, 95% 1.052-9.012) and respiratory infection as a trigger (adjusted : 3.926, 95% 1.141-13.510) were independent risk factors for the progression to MC, while intravenous immunoglobulin (IVIg) treatment prior to the mechanical ventilation (adjusted : 0.253, 95% 0.087-0.732) was a protective factor. The prognosis did not significantly differ between the patients with and without MC during the MG course, with a total of 45 (52.3%) patients reaching post-intervention status better than minimal manifestations at the last follow-up. When treating the patients with MG with acute dyspnea, the clinicians should be aware of the risk factors of progression to MC, such as early-onset MG and respiratory infection. IVIg is an effective treatment. With proper immunosuppressive therapy, this group of patients had an overall good long-term prognosis.
危及生命的重症肌无力危象（MC）发生于10% - 20%的重症肌无力（MG）患者中。识别MG急性加重患者进展为MC的预测因素及预后很重要。本研究旨在探讨急性起病呼吸困难的MG患者进展为MC的预测因素及其短期和长期预后。本研究为回顾性队列研究。我们使用单因素和多因素分析，收集并分析了一个中心10年间所有急性呼吸困难的MG患者的数据。纳入了86例MG患者。在他们首次急性呼吸困难发作中，36例（41.9%）最终进展为MC。多因素分析显示，早发型MG（校正后：3.079，95%置信区间1.052 - 9.012）和作为诱因的呼吸道感染（校正后：3.926，95%置信区间1.141 - 13.510）是进展为MC的独立危险因素，而在机械通气前进行静脉注射免疫球蛋白（IVIg）治疗（校正后：0.253，95%置信区间0.087 - 0.732）是保护因素。在MG病程中，有MC和无MC的患者预后无显著差异，共有45例（52.
