Shi Jiayu, Tan Ying, Huang Yangyu, Li Ke, Yan Jingwen, Guan Yuzhou, Zhang Li
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Department of Respiratory and Critical Care, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Front Neurol. 2022 Apr 7;13:858628. doi: 10.3389/fneur.2022.858628. eCollection 2022.
Neurological immune-related adverse events (nirAEs) are rare toxicities of immune-checkpoint inhibitors (ICI). With the increase use of ICIs, incidence of nirAEs is growing, among which ICI related MG (irMG) is causing high fatality rate. Given the limited evidence, data from a large cohort of patients with irMG is needed to aid in recognition and management of this fatal complication.
This study aimed to summarize clinical characteristics of irMG and explore predictors of irMG clinical outcome.
We summarized our institution's patients who were diagnosed as irMG between Sep 2019 and Oct 2021. We systematically reviewed the literature through Oct 2021 to identify all similar reported patients who met inclusion criteria. As the control group, patients with idiopathic MG were used. We collected data on clinical features, management, and outcomes of both irMG and idioMG cases. Further statistical analysis was conducted.
Sixty three irMG patients and 380 idioMG patients were included in the final analysis. For irMG patients, six were from our institution while the rest 57 were from reported cases. The average age of irMG patients is 70.16 years old. Forty three were male. Average time from first ICI injection to symptom onset was 5.500 weeks. Eleven patients had a past history of MG. Higher MGFA classification and higher QMGS rates were observed in irMG patients compared to idioMG patients. For complication, more irMG patients had myositis or myocarditis overlapping compared to idioMG patients. The most commonly used treatment was corticosteroids for both idioMG and irMG. Twenty one patients (35%) with irMG had unfavorable disease outcome. Single variate and multivariate binary logistic regression proved that association with myocarditis, high MGFA classification or QMGS rates at first visit were negatively related to disease outcome in irMG patients.
irMG is a life-threatening adverse event. irMG has unique clinical manifestations and clinical outcome compared to idioMG. When suspicious, early evaluation of MGFA classification, QMGS rates and myositis/myocarditis evaluation are recommended.
神经免疫相关不良事件（nirAEs）是免疫检查点抑制剂（ICI）罕见的毒性反应。随着ICI使用的增加，nirAEs的发生率不断上升，其中ICI相关重症肌无力（irMG）的致死率较高。鉴于证据有限，需要大量irMG患者的数据来帮助识别和管理这种致命并发症。
本研究旨在总结irMG的临床特征，并探索irMG临床结局的预测因素。
我们总结了2019年9月至2021年10月间在我院被诊断为irMG的患者。我们系统回顾了截至2021年10月的文献，以确定所有符合纳入标准的类似报道患者。作为对照组，使用特发性重症肌无力患者。我们收集了irMG和特发性重症肌无力病例的临床特征、治疗和结局数据，并进行了进一步的统计分析。
最终分析纳入了63例irMG患者和380例特发性重症肌无力患者。对于irMG患者，6例来自我院，其余57例来自报道病例。irMG患者的平均年龄为70.16岁。43例为男性。从首次注射ICI到症状出现的平均时间为5.500周。11例患者有重症肌无力病史。与特发性重症肌无力患者相比，irMG患者的MGFA分类更高，QMGS率更高。对于并发症，与特发性重症肌无力患者相比，更多的irMG患者合并肌炎或心肌炎。特发性重症肌无力和irMG最常用的治疗方法都是皮质类固醇。21例（35%）irMG患者疾病结局不佳。单变量和多变量二元逻辑回归证明，首次就诊时合并心肌炎、MGFA分类高或QMGS率高与irMG患者的疾病结局呈负相关。
irMG是一种危及生命的不良事件。与特发性重症肌无力相比，irMG具有独特的临床表现和临床结局。可疑时，建议早期评估MGFA分类、QMGS率以及肌炎/心肌炎。
