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増大特集 筋疾患update
文献概要
はじめに
自己免疫機序により筋線維が障害される筋炎は，多発筋炎（polymyositis：PM），皮膚筋炎（dermatomyositis：DM），封入体筋炎の3つに大別されるが，そのほかにもさまざまな病態機序を背景に持つ疾患が含まれており，臨床像においても病態機序においても均一ではない1,2)。現在，PMとDMは筋病理所見や病態機序から異なる疾患として位置づけられている。しかし，厳密な病理所見をもとにしたPMの診断基準によると，PMと診断できる症例は稀であり，多くは非特異的筋炎やpossible myositisに含まれてしまう問題点もある3)。一方，自己抗体の立場からみた場合はPMとDMは連続した疾患であり，いまだにPM/DMという記載が一般的である4)。これは，筋炎はあくまでも臓器病変の1つであり，PM/DMは自己免疫が介在した全身性炎症疾患という考え方である。
本稿では，筋炎で検出される自己抗体とその臨床的意義を中心に解説する。
自己免疫機序により筋線維が障害される筋炎は，多発筋炎（polymyositis：PM），皮膚筋炎（dermatomyositis：DM），封入体筋炎の3つに大別されるが，そのほかにもさまざまな病態機序を背景に持つ疾患が含まれており，臨床像においても病態機序においても均一ではない1,2)。現在，PMとDMは筋病理所見や病態機序から異なる疾患として位置づけられている。しかし，厳密な病理所見をもとにしたPMの診断基準によると，PMと診断できる症例は稀であり，多くは非特異的筋炎やpossible myositisに含まれてしまう問題点もある3)。一方，自己抗体の立場からみた場合はPMとDMは連続した疾患であり，いまだにPM/DMという記載が一般的である4)。これは，筋炎はあくまでも臓器病変の1つであり，PM/DMは自己免疫が介在した全身性炎症疾患という考え方である。
本稿では，筋炎で検出される自己抗体とその臨床的意義を中心に解説する。
参考文献
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