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Exacerbation of myasthenia gravis following corticosteroid treatment: what is the evidence? A systematic review
Journal of Neurology ( IF 4.8 ) Pub Date : 2020-10-16 , DOI: 10.1007/s00415-020-10264-0
Itay Lotan 1, 2, 3 , Mark A Hellmann 1, 2, 3 , Adi Wilf-Yarkoni 1, 2, 3 , Israel Steiner 1, 3
Affiliation  

Corticosteroids (CS) are among the most widely- used immunosuppressive agents for immune-mediated conditions, including myasthenia gravis (MG). While their effectiveness in MG is documented and supported in the clinical practice over several decades, one of the main drawbacks of treatment results from the notion that MG patients may experience symptom worsening following CS treatment initiation. This may lead to the administration of lower than necessary doses of CS for the disorder, or even avoiding them altogether. As a consequence, some patients may not receive the optimal treatment to control their disease. In the present review, we analyzed 27 relevant publications and determined the prevalence of clinical exacerbation following CS treatment, its’ severity and relation to the type and dose of CS. The rate of MG exacerbation is highest with the administration of cortisone, intermediate with prednisone, and lowest with methylprednisolone. High dose daily or alternate-day prednisone is associated with exacerbation more frequently than low-dose treatment, but most exacerbations are of mild to moderate severity. Other factors related to increased risk of an initial exacerbation include older age, generalized MG, bulbar symptoms, disease severity, presence of thymoma, and thymectomy. However, the current information is based mostly on heterogeneous studies of low quality, and prospective clinical trials designed to compare between the various agents and doses and assess the rate and severity of the exacerbation by a unified scale are warranted.



中文翻译:

皮质类固醇治疗后重症肌无力恶化:证据是什么?系统评价

皮质类固醇(CS)是用于免疫介导疾病(包括重症肌无力(MG))最广泛使用的免疫抑制剂之一。虽然它们在 MG 中的有效性已在数十年的临床实践中得到证实和支持,但治疗的主要缺点之一是认为 MG 患者在开始 CS 治疗后可能会出现症状恶化。这可能导致对疾病给予低于必要剂量的 CS,甚至完全避免使用它们。因此,一些患者可能无法接受最佳治疗来控制他们的疾病。在本综述中,我们分析了 27 篇相关出版物,并确定了 CS 治疗后临床恶化的患病率、严重程度以及与 CS 类型和剂量的关系。MG 恶化率在可的松组中最高,在强的松组中度最高,在甲基强的松龙组中最低。每日大剂量或隔日泼尼松比低剂量治疗更频繁地与急性加重相关,但大多数急性加重为轻度至中度严重程度。与初始恶化风险增加相关的其他因素包括年龄较大、全身性 MG、延髓症状、疾病严重程度、胸腺瘤的存在和胸腺切除术。然而,目前的信息主要基于低质量的异质性研究,有必要进行旨在比较各种药物和剂量并通过统一的尺度评估恶化的速度和严重程度的前瞻性临床试验。每日大剂量或隔日泼尼松比低剂量治疗更频繁地与急性加重相关,但大多数急性加重为轻度至中度严重程度。与初始恶化风险增加相关的其他因素包括年龄较大、全身性 MG、延髓症状、疾病严重程度、胸腺瘤的存在和胸腺切除术。然而,目前的信息主要基于低质量的异质性研究,有必要进行旨在比较各种药物和剂量并通过统一的尺度评估恶化的速度和严重程度的前瞻性临床试验。每日大剂量或隔日泼尼松比低剂量治疗更频繁地与急性加重相关,但大多数急性加重为轻度至中度严重程度。与初始恶化风险增加相关的其他因素包括年龄较大、全身性 MG、延髓症状、疾病严重程度、胸腺瘤的存在和胸腺切除术。然而,目前的信息主要基于低质量的异质性研究,有必要进行旨在比较各种药物和剂量并通过统一的尺度评估恶化的速度和严重程度的前瞻性临床试验。与初始恶化风险增加相关的其他因素包括年龄较大、全身性 MG、延髓症状、疾病严重程度、胸腺瘤的存在和胸腺切除术。然而,目前的信息主要基于低质量的异质性研究,有必要进行旨在比较各种药物和剂量并通过统一的尺度评估恶化的速度和严重程度的前瞻性临床试验。与初始恶化风险增加相关的其他因素包括年龄较大、全身性 MG、延髓症状、疾病严重程度、胸腺瘤的存在和胸腺切除术。然而,目前的信息主要基于低质量的异质性研究,有必要进行旨在比较各种药物和剂量并通过统一的尺度评估恶化的速度和严重程度的前瞻性临床试验。

更新日期:2020-10-17
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