A new version of the Japanese clinical guidelines for myasthenia gravis is under development. The current version was published in 2014, in which the top priority in treatment was to maintain patients’ health-related quality of life. The treatment goal was to achieve a post-intervention 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. The guidelines recommend minimizing the oral prednisolone dose, starting calcineurin inhibitors early in the course of treatment, and effectively treating patients with an early fast-acting treatment regimen. Since the release of the 2014 version, the therapeutic tendencies in myasthenia gravis have been gradually changing in Japan, with an observed increase in the number of patients that achieved the treatment goal. Therefore, it is conceivable that the rationale of the 2014 guidelines was appropriate to treat patients with myasthenia gravis and that it should be taken over by the new guidelines. Meanwhile, the anti-complement antibody (eculizumab) has been approved, and although off-label in Japan, the effect of the anti-CD20 antibody rituximab has been reported repeatedly. Many other agents are currently undergoing clinical trials. In the new guidelines, the treatment strategy should be demonstrated clearly, including these most recent developments.
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