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Title 好酸球性多発血管炎性肉芽腫症 (EGPA) における診断と治療の進歩
Subtitle 綜説
Authors 谷口正実, 上出庸介, 中村祐人, 渡井健太郎, 福冨友馬, 関谷潔史
Authors (kana)
Organization 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 73
Number 1
Page 26-33
Year/Month 2024 /
Article 報告
Publisher 日本アレルギー学会
Abstract 「1. はじめに. 診断における進歩と課題」好酸球性多発血管炎性肉芽腫症(eosinophilic granulomatosis with polyangiitis, EGPA)は, 著明な好酸球増加, 重症喘息, および全身性血管炎を特徴とするまれな疾患である. 小児期発症は極めてまれであり, 30歳から65歳で発症しやすく, 喘息先行期間は1〜20年で, 男女比は1:2である. 難病指定されており, 2022年度では6723人が登録されている. 成人喘息患者の約0.35%がEGPAであることが判明しており, 国内患者数は1.5万人程度と推定される. 本稿では, 紙面の都合により, 診断と治療における最近の進歩を中心に概説する. EGPAにおける確実な診断基準はない. その理由は, 診断の前提となる血管炎の症状や程度が多彩であり, 血管炎そのものを明確に診断/定義することが困難なためである.
Practice 臨床医学：内科系
Keywords eosinophil, eosinophilic granulomatosis with polyangiitis(EGPA), intravenous immunoglobulin(IVIG), mepolizumab, nonsteroidal anti-inflammatory drug-exacerbated respiratory disease(N-ERD)
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