臨床検査68巻6号

2024年06月発行

今月の特集 免疫・アレルギー性肺疾患と検査 疾患各論

好酸球性多発血管炎性肉芽腫症（EGPA）

著者： 谷口正実¹ 上出庸介¹ 関谷潔史¹

所属機関： ¹国立病院機構相模原病院臨床研究センター

ページ範囲：P.734 - P.741

Point

●好酸球性多発血管炎性肉芽腫症（EGPA）は，成人発症喘息と好酸球性副鼻腔炎が数年先行した後に，著明な好酸球増加とともに小動脈を病変の主座とする全身性血管炎を発症する原因不明のまれな疾患で，病態の本質は全身臓器の虚血と好酸球性炎症である．

●全例で好酸球性気道炎症病態が先行するため特発性好酸球性肺炎などとの鑑別が，また発症時に末梢血好酸球数が1,000/μL以上を示すため好酸球増多疾患との鑑別が必要となる．

●発症急性期には末梢血好酸球は40％以上を示し，臓器虚血指標〔乳酸脱水素酵素（LDH），クレアチンキナーゼ（CK）〕が増加し，2/3の例で血清免疫グロブリンE（IgE），血小板，リウマチ因子が上昇し，1/3でp-ANCAが陽性化する．

●多発単神経炎による痺れや麻痺をきたしやすく，心臓障害（心不全と致死的不整脈）や虚血性腸障害も約4割で認め，急性期は急激に悪化しやすく，迅速な諸検査による臓器障害の把握と早期の治療開始が重要である．

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