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Myasthenic crisis in thymoma-associated myasthenia gravis: a multicenter retrospective cohort study

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机构： [1]Huashan Rare disease center, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China [2]National Center for Neurological Disorders, Shanghai 200040, China [3]Department of Neurology, Tangdu Hospital, The Air Force Medical University, Xi’an 710000, China [4]Department of Neurology, Fujian Medical University Union Hospital, Institute of Clinical Neurology, Fujian Medical University, Fuzhou 350001, China [5]Department of Neurology, Sichuan Provincial People’s Hospital, University of Electronic Science and Technology of China, Chengdu 610072, China [6]Department of Neurology, Wuhan No.1 Hospital, Wuhan 430030, China [7]Department of Neurology, The First People’s Hospital of Changzhou, Third Affiliated Hospital of Soochow University, Changzhou 213003, China [8]Department of Neurology, Nanjing First Hospital, Nanjing Medical University, Nanjing 210006, China [9]Department of Neurology, Zhongnan Hospital of Wuhan University, Wuhan 430071, China [10]Department of Neurology, The First Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou 510405, China

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关键词： Myasthenia gravis Myasthenic crisis Thymoma Disease progression

摘要：

Thymoma-associated myasthenia gravis (TMG) had more severe symptoms and worse prognoses in comparison to non-thymoma-associated MG. Thymoma recurrence was frequently associated with transient worsening of MG and even acute respiratory failure, namely myasthenic crisis (MC). However, little is known about the clinical features and outcomes of MC in thymoma-associated MG patients. We performed a retrospective cohort study in MG patients recruited from 9 independent tertiary neuromuscular centers in China from Jan 2015, through Oct 2022. Overall, 156 MC from 149 MG patients with positive anti-acetylcholine receptor (AChR) antibodies were finally analyzed. Next, these patients were divided into two subgroups: the TMG group (n = 60 MCs, 58 patients) and the non-thymoma-associated MG group (n = 96 MCs, 91 patients). Compared with non-thymoma-associated MG, TMG patients had a significantly shorter disease duration from symptom onset to the crisis (17.95±40.9 vs 51.31±60.61 months, P<0.0001), a larger proportion of MGFA IVa as the initial onset clinical classification (6.67% vs 0, P = 0.0205), and a longer hospital stay (39.24±22.09 [6-111] vs. 33.2 ± 23.42 days [7-120]; P = 0.0317) during the crisis. Within the TMG group, the hospital stay was significantly longer in patients with unresected thymoma compared to that in postoperative myasthenic crisis (POMC) (47.68±24.9 [6-111] vs. 34.21±18.87 days [12-82]; P = 0.0257). Early identification of the MG categories may provide some hints in tailoring therapeutic strategies to improve the prognosis.Copyright © 2023 Elsevier B.V. All rights reserved.

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出版当年[2023]版：

大类 | 4 区医学

小类 | 4 区临床神经病学 4 区神经科学

最新[2023]版：

大类 | 4 区医学

小类 | 4 区临床神经病学 4 区神经科学

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出版当年[2023]版：

Q2 CLINICAL NEUROLOGY Q3 NEUROSCIENCES

最新[2023]版：

Q2 CLINICAL NEUROLOGY Q3 NEUROSCIENCES

影响因子： 2.7 最新[2023版] 3.2 最新五年平均 2.7 出版当年[2023版] 3.2 出版当年五年平均 2.8 出版前一年[2022版]

第一作者：

第一作者机构： [1]Huashan Rare disease center, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China [2]National Center for Neurological Disorders, Shanghai 200040, China

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通讯作者：

通讯机构： [1]Huashan Rare disease center, Department of Neurology, Huashan Hospital Fudan University, Shanghai 200040, China [2]National Center for Neurological Disorders, Shanghai 200040, China

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Myasthenic crisis in thymoma-associated myasthenia gravis: a multicenter retrospective cohort study

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