ステロイド・IVIgで筋力回復が不良な皮膚筋炎・壊死性筋症—全身を診察して的確な診断，評価のうえで治療を行う

Treatment of Dermatomyositis and Immune-Mediated Necrotizing Myopathy with Poor Muscle Recovery with Steroids and IVIg: Therapeutic Strategies for Refractory Inflammatory Myositis

筋炎は封入体筋炎を除いて治療可能な疾患である。副腎皮質ステロイドなどの免疫治療を行うが，検出される筋炎特異抗体によって臨床型や治療反応性，予後が異なる。そのために的確な診断と重症度に適した治療法を導入する必要がある。効果は血清クレアチンキナーゼ値や筋力改善，画像などで評価する。ステロイド抵抗性と判断した場合は早期に免疫抑制薬を開始し，その効果が表れるまでは免疫グロブリン大量静注療法を適宜追加することを勧める。

Abstract

The majority of inflammatory myositis cases can be cured by immunomodulatory therapies. We recently observed that the phenotype and response to therapies differed according to myositis-specific autoantibodies; therefore, it is essential to select a suitable therapy after thoroughly evaluating the autoantibody, clinical severity, and complications. In some cases, the symptoms can be controlled by steroid monotherapy, but some cases exhibit steroid resistance and require other therapies. We recommend intensive treatment involving the addition of immunosuppressive agents in the early stage and repeated intravenous administration of immunoglobulin therapy in cases of refractory myositis, such as immune-mediated necrotizing myopathy.