筋炎と壊死性筋症の最新治療

Immunotherapy for Inflammatory Myopathies

炎症性筋疾患は筋肉の炎症に加えて，皮膚，肺，心臓，関節にも障害を呈し得る疾患である。炎症性筋疾患は希少疾患であり，その病像や病態がさまざまであることから治療の標準化が難しい。治療の第一選択は副腎皮質ステロイド薬であり，治療抵抗例などに対して免疫抑制薬や免疫グロブリンが併用される。数多くの生物学的製剤の臨床試験が現在進行中であり，今後の炎症性筋疾患の治療選択肢となることが期待される。

Abstract

Inflammatory myopathies are heterogeneous disorders characterized by muscle inflammation. They are frequently accompanied by extra-muscular manifestations that affect the skin, lungs, heart, and joints. Owing to its low prevalence, wide phenotypic heterogeneity, and variable disease course, it is difficult to make clear recommendations for the treatment of inflammatory myopathies. Corticosteroids are administered as first-line treatment based on clinical experience rather than controlled trial findings. Empirically, addition of an immunosuppressive drug might offer a steroid-sparing effect or an additional benefit. Administration of intravenous immunoglobulins has been shown to be effective as second-line treatment. Recently, there has been a growing interest in assessing the potential of several biological agents in the treatment of inflammatory myopathies. There are multiple ongoing clinical trials that will lead to more treatment options for inflammatory myopathies.