ANCA関連血管炎の基礎病態

Abstract

ANCA関連血管炎（ANCA-associated vasculitis： AAV）は，抗好中球細胞質抗体（anti-neutrophil cytoplasmic antibody：ANCA）の出現とともに，全身の小型血管炎を発症する自己免疫疾患である．AAVは他の自己免疫疾患と同様，何らかの遺伝要因を背景に，感染等の環境要因が加わり発症すると考えられている．その機序は，①好中球のプライミング，②プライミングされた好中球へのANCAの結合，③ANCAの結合による好中球の過剰な活性化と好中球細胞外トラップ（neutrophil extracellular traps： NETs）の形成，④活性化好中球による血管内皮傷害とNETsの除去障害の4つの段階に分けられる．本総説ではAAVの基礎病態をまとめ，その中において重要な役割を果たしているNETsを標的としたAAVの新規治療戦略を紹介する．

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