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Title	2. 好酸球性多発血管炎性肉芽腫症
Subtitle	専門医のためのアレルギー学講座 38. アレルギー性肺疾患 (類縁疾患) の基本から最新情報まで
Authors	谷口正実1)2), 関谷潔史2), 上出庸介2), 福冨友馬2), 渡井健太郎2), 濱田祐斗2), 中村祐人2), 劉楷2), 藤田教寛2), 矢野光一2), 岩田真紀2), 永山貴紗子2), 森晶夫2)
Authors (kana)
Organization	1)湘南鎌倉総合病院免疫・アレルギーセンター, 2)国立病院機構相模原病院臨床研究センター
Journal	アレルギー
Volume	69
Number	5
Page	293-318
Year/Month	2020 /
Article	報告
Publisher	日本アレルギー学会
Abstract	「はじめに」好酸球性多発血管炎性肉芽腫症 (Eosinophilic Granulomatosis with Polyangitis, EGPA) は, 好酸球増多が目立つ重症喘息が数年先行した後に発症する全身性の小血管炎で2012年にChurg-Strauss症候群からEGPAに名称変更された. 顕微鏡的多発血管炎 (microscopic polyangiitis, MPA) や多発血管炎性肉芽腫症 (granulomatosis with polyangiitis, GPA) とともに抗好中球細胞質抗体 (ANCA) 関連血管炎に属するが, ANCAの陽性率は3割程度しかなく, 陽性群と陰性群の比較では, 臨床像だけでなく遺伝的背景や治療反応性も異なる可能性が指摘されている. 基本症状は, 数年先行する好酸球性下気道炎症 (100%) と発症時の四肢末梢のしびれと麻痺を主訴とする多発性単神経炎 (90%以上) である.
Practice	臨床医学：内科系
Keywords	EGPA, mepolizumab, severe asthma

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