Service de Neurologie, Centre de Référence Neuromusculaire Nord/Est/Ile de France, Hôpital de Hautepierre, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
ERN EURO-NMD, Paris, France.
J Neurol. 2023 Dec;270(12):5819-5826. doi: 10.1007/s00415-023-11925-6. Epub 2023 Aug 17.
Myasthenia gravis (MG) is an autoimmune disease treated with acetylcholinesterase inhibitors and immunosuppressant/immunomodulatory drugs. MG is frequently diagnosed in elderly patients, a fragile population in which treatment adverse effects (TAE) have not been evaluated until now.
We retrospectively analysed the files of all MG patients with disease onset after age 70 years in four French University Hospitals, including clinical, electrophysiological, biological, and treatment data, with an emphasis on TAE. MG outcomes were assessed using the Myasthenia Gravis Foundation of America (MGFA) status scale.
We included 138 patients (59% of men) with a mean follow-up of 4.5 years (range 1-19). Mean age at diagnosis was 78 years (70-93). Anti-acetylcholine receptor antibodies were found in 87% of cases, electrophysiological abnormalities in 82%, and thymoma in 10%. MG outcome was good in a majority of cases, with 76% of treated patients presenting with alleviated symptoms at follow-up. TAE were observed in 41% of patients, including severe TAE in 14% of cases. Seven patients (5.1%) died, including four (2.9%) from MG-related respiratory failure, and three (2.2%) from MG treatment-related complications, i.e., sepsis in 2 cases and brain toxoplasmosis in 1 case. TAE were observed in 53% of patients treated with azathioprine, 23% of patients treated with corticosteroids, and 15% of patients treated with mycophenolate mofetil.
This retrospective study demonstrates MG in the elderly presents with a significant iatrogenic risk, including fatal immunosuppressant-related infections.
重症肌无力(MG)是一种自身免疫性疾病,采用乙酰胆碱酯酶抑制剂和免疫抑制剂/免疫调节剂治疗。MG 常发生于老年患者,该人群为脆弱群体,目前尚未对其治疗不良反应(TAE)进行评估。
我们回顾性分析了法国四家大学附属医院中所有发病年龄在 70 岁以上的 MG 患者的病历,包括临床、电生理、生物学和治疗数据,重点关注 TAE。MG 结局采用美国重症肌无力基金会(MGFA)状态量表进行评估。
我们纳入了 138 例患者(59%为男性),平均随访时间为 4.5 年(1-19 年)。诊断时的平均年龄为 78 岁(70-93 岁)。87%的病例存在抗乙酰胆碱受体抗体,82%存在电生理异常,10%存在胸腺瘤。大多数患者的 MG 结局良好,76%的治疗患者在随访时症状缓解。41%的患者出现 TAE,其中 14%为严重 TAE。7 例(5.1%)患者死亡,其中 4 例(2.9%)死于与 MG 相关的呼吸衰竭,3 例(2.2%)死于与 MG 治疗相关的并发症,即 2 例脓毒症和 1 例脑弓形体病。接受硫唑嘌呤治疗的患者中有 53%出现 TAE,接受皮质类固醇治疗的患者中有 23%出现 TAE,接受吗替麦考酚酯治疗的患者中有 15%出现 TAE。
这项回顾性研究表明,老年 MG 患者存在显著的医源性风险,包括致命的免疫抑制剂相关感染。