BRAIN and NERVE－神経研究の進歩74巻5号

2022年05月発行

増大特集 次の一手—神経筋疾患難治例をどのように治療するか

ステロイド・IVIgで筋力回復が不良な皮膚筋炎・壊死性筋症—難治性の皮膚筋炎・壊死性筋症

著者： 杉江和馬¹

所属機関： ¹奈良県立医科大学脳神経内科学講座

ページ範囲：P.537 - P.544

文献概要

特発性炎症性筋疾患である皮膚筋炎と免疫介在性壊死性筋症は，臨床病理学的特徴が異なり区別される。いずれもステロイド治療が第一選択で，免疫抑制薬や免疫グロブリン大量静注療法が適宜使用される。少数例ながらこれらの治療への抵抗性を示すため，各症例における丹念な四肢・体幹の筋力評価に加えて，全身炎症所見や筋病理所見，骨格筋画像，悪性腫瘍や間質性肺疾患などの合併症を勘案して，治療計画を立てる必要がある。現状では，難治例に対する治療戦略は十分確立しておらず，使用可能な治療薬が限られていることからも，今後の治療法の開発が求められる。

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