Department of Neurology, School of Medicine, International University of Health and Welfare, Narita, Japan.
Department of Neurology, Hanamaki General Hospital, Hanamaki, Japan.
Ann N Y Acad Sci. 2018 Feb;1413(1):35-40. doi: 10.1111/nyas.13544. Epub 2018 Jan 28.
According to the 2014 Japanese clinical guidelines for myasthenia gravis, the most important priority in treatment is maintaining patients' health-related quality of life. Therefore, the initial treatment goal is defined as maintaining a postintervention status of minimal manifestations or better (according to the Myasthenia Gravis Foundation of America classification) with an oral prednisolone dose of 5 mg/day or less. Every effort should be made to attain this level as rapidly as possible. To achieve this goal, the guidelines recommend minimizing the oral prednisolone dose, starting calcineurin inhibitors early in the course of treatment, using intravenous methylprednisolone infusion judiciously (often combined with plasma exchange/plasmapheresis or intravenous immunoglobulin), and effectively treating patients with an early, fast-acting treatment strategy. The early, fast-acting treatment strategy enables more frequent and earlier attainment of the initial goal than other strategies. Thymectomy is considered an option for treating nonthymomatous early-onset myasthenia gravis in patients with antiacetylcholine receptor antibodies and thymic hyperplasia in the early stages of the disease.
根据 2014 年日本重症肌无力临床指南，治疗的最重要优先事项是维持患者的健康相关生活质量。因此，初始治疗目标定义为用 5 毫克/天或更少的口服泼尼松龙剂量维持最小表现或更好的干预后状态（根据美国重症肌无力基金会分类）。应尽一切努力尽快达到这一水平。为了实现这一目标，指南建议尽量减少口服泼尼松龙剂量，在治疗过程早期开始使用钙调神经磷酸酶抑制剂，明智地使用静脉甲基泼尼松龙输注（常与血浆置换/血浆分离或静脉注射免疫球蛋白联合使用），并采用早期、快速作用的治疗策略有效地治疗患者。与其他策略相比，早期、快速作用的治疗策略能更频繁、更早地达到初始目标。胸腺切除术被认为是治疗早期发病的抗乙酰胆碱受体抗体阳性且疾病早期存在胸腺瘤样增生的非胸腺瘤性重症肌无力患者的一种选择。
