アブストラクト
| Title | 免疫介在性壊死性ミオパチー |
|---|---|
| Subtitle | 特集・第65回日本小児神経学会学術集会 シンポジウム13 : 小児免疫性神経疾患の臨床と病態update |
| Authors | 漆葉章典 |
| Authors (kana) | |
| Organization | 東京都立神経病院脳神経内科 |
| Journal | 脳と発達 |
| Volume | 56 |
| Number | 4 |
| Page | 290-294 |
| Year/Month | 2024 / 7 |
| Article | 報告 |
| Publisher | 日本小児神経学会 |
| Abstract | 「要旨」 免疫介在性壊死性ミオパチーは自己免疫性筋炎の一型で, 筋病理学的に壊死・再生過程を主要所見とする. 特異自己抗体として抗signal recognition particle(SRP)抗体と抗3-hydroxy-3-methylglutaryl-coenzyme A reductase(HMGCR)抗体が同定されている. 高度の筋線維壊死を反映し, 血清クレアチンキナーゼ(CK)値は著しい高値を示す. 主症状は亜急性経過の四肢近位筋力低下で, 筋力低下はしばしば高度である. 上肢挙上困難など上肢帯の筋力低下が病初期から目立つ例もある. 近年, 小児例の報告により, 小児筋炎の主要病型の一つであることがわかってきた. 小児例では経過が一見, 慢性的に見えることがあり, 筋病理学的に壊死・再生過程を主体とする点で類似する筋ジストロフィーとの鑑別に難渋することがある. 免疫介在性壊死性ミオパチーの見落としは治療方針や予後に大きく影響するため, 鑑別診断を検討する際にはその可能性を積極的に考慮すべきである. 免疫介在性壊死性ミオパチーの可能性を想起するポイントとして, 「亜急性発症」, 「血清CK値1,000IU/L以上」, 「筋ジストロフィーと暫定診断されているが, 分子診断は未確定」という三点が挙げられる. これらを満たす例では, 抗SRP抗体や抗HMGCR抗体を含む自己抗体測定を行うことが望まれる. 治療にはステロイドに加え, 免疫抑制剤や免疫グロブリンなどが組み合わせられる. |
| Practice | 臨床医学：内科系 |
| Keywords | 抗SRP抗体, 抗HMGCR抗体, 亜急性発症, 高クレアチンキナーゼ血症, 筋ジストロフィー |
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