神経治療学
Online ISSN : 2189-7824
Print ISSN : 0916-8443
ISSN-L : 2189-7824
教育講演
重症筋無力症の標準的治療と新規治療
槍沢 公明
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ジャーナル フリー

2019 年 36 巻 4 号 p. 349-353

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Achieving complete stable remission (CSR) may be an ideal goal, but it is uncommon for MG patients, and the rate of CSR cannot be increased at least by presently available treatment. Early achievement of minimal manifestations (MM) or better status on prednisolone at ≤5mg/day (MM–or–better–5mg) is now proposed as a practical and initial goal, and which is another way to say “early return to a normal lifestyle without complications from steroids”. However, the percentage of patients who achieved this goal was not high in our subjects of surveys in 2010 and 2012, and they were treated mainly by chronic oral immune therapies based on corticosteroids. It was also shown that longer treatment with a higher dose of oral steroids does not ensure better outcome. These indicate that changes in treatments are needed to further increase treatment success.

To achieve MM–or–better–5 mg status early, it is better to use steroid–sparing agents, such as calcineurin inhibitors, during the early stages of treatment. To reduce the steroid dose and achieve early improvement of symptoms, non–oral fast–acting treatment such as high–dose methylprednisolone, plasmapheresis and/or IVIG can be more aggressively used. They can be repeated as needed during both the early stages and chronic stages of treatment to achieve early improvement with sparing oral steroids. If the MM status cannot be maintained, a fast–acting treatment can be repeated as maintenance therapy to avoid dose–up of oral steroids. Such treatment approaches are now proposed in the Japanese guidelines and named as early fast–acting treatment strategy (EFT). It is shown that EFT can promote early achievement of MM–or–better–5mg lasting more than 6 months. In an attempt to achieve early goal by EFT, dosing regimens of oral steroids (low–dose or high–dose) produce no difference in the outcome. If the frequency of fast–acting treatment is too high and not decreased for years or if the effects are insufficient, molecular target therapies (new arms) now can be an effective next step.

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