Rationale for the clinical guidelines for myasthenia gravis in Japan

Annals of the New York Academy of Sciences - Tập 1413 Số 1 - Trang 35-40 - 2018
Hiroyuki Murai1, Kimiaki Utsugisawa2, Yuriko Nagane2, Shigeaki Suzuki3, Tomihiro Imai4, Masakatsu Motomura5
1Department of Neurology, School of Medicine, International University of Health and Welfare, Narita, Japan
2Department of Neurology, Hanamaki General Hospital, Hanamaki, Japan
3Department of Neurology, Keio University School of Medicine, Tokyo, Japan
4Department of Occupational Therapy, Sapporo Medical University School of Health Sciences, Sapporo, Japan
5Medical Engineering Course, Department of Engineering, The Faculty of Engineering, Nagasaki Institute of Applied Science, Nagasaki, Japan

Tóm tắt

AbstractAccording 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.

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10.1056/NEJM199406233302507

10.1002/mus.880171208

10.1002/ana.20341

10.1002/ana.22312

10.1016/j.jns.2011.03.004

10.1111/cen3.12180

10.1002/mus.24213

10.1212/WNL.55.1.16

10.1002/mus.20950

10.1002/mus.21053

10.1136/bmjopen-2011-000313

10.1002/mus.23398

10.1212/WNL.0000000000002790

10.1002/mus.24438

10.1002/mus.21462

10.1111/j.1600-0404.1998.tb05968.x

10.1159/000062987

10.1002/mus.25397

10.1056/NEJMoa1602489