筋疾患の治療の進歩

抄録

This article reviewed the representative papers of treatment of immune–mediated muscular disorder published in 2018. We focused on molecular target drugs for treatment of myasthenia gravis and inflammatory myopathies. Eculizumab was approved by the Japanese government for the treatment of acetylcholine receptor antibodies–positive refractory MG in December 2017. Eculizumab is expected to have treatment benefits for refractory and generalized MG patients, although Eculizumab is the most expensive treatment for MG in Japan. Neuromuscular experts should keep the appropriate indication of eculizumab for refractory MG in mind. Rituximab has shown to be a remarkable effective drug for MG, especially seropositive for autoantibodies against muscle–specific kinase. Unfortunately, rituximab is not available for MG treatment. Randomized phase 2 clinical study demonstrated that belimumab did not have significant efficacy compared with placebo in generalized MG. In contrast, abatacept and infliximab were well tolerated and potentially effective for treatment of refractory polymyositis and dermatomyositis.