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Title 慢性炎症性脱髄性多発神経炎
Subtitle 特集 神経難病の今 〜疫学・成因・治療の研究最前線〜
Authors 飯島正博
Authors (kana)
Organization 名古屋大学医学部附属病院先端医療開発部
Journal Pharma Medica
Volume 39
Number 3
Page 49-53
Year/Month 2021 / 3
Article 報告
Publisher メディカルレビュー社
Abstract 「はじめに」 慢性炎症性脱髄性多発神経炎(chronic inflammatory demyelinating polyneuropathy: CIDP)は脱髄を特徴とする免疫介在性ニューロパチーであり, 全年齢層で発症しうる後天性疾患である. 慢性の経過を呈し, 治療を行わなければ病勢は長期間持続しうる. また, 四肢の脱力, 感覚鈍麻が主症状で, 稀ではあるが一部に脳神経症状や感覚性運動失調, 振戦を伴う例がある. 病因は今のところ不明で, 髄鞘もしくは傍絞輪部を標的とする免疫応答が想定されるが, 液性, 細胞性両者の免疫機序の関与が指摘されるなど, 病態の多様性が示唆される. 急性期治療にはステロイド, 免疫グロブリン大量療法, 血漿浄化療法がファーストライン治療として確立している. ただし, 発症後の治療介入が遅れることで, 脱髄から不可逆性の軸索変性に至るおそれがある.
Practice 薬学
Keywords 慢性炎症性脱髄性多発神経炎 (CIDP), IgG4, NF155, CNTN1, 治療
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