Expert Consensus on the Diagnosis and Treatment of Hereditary Angioedema in China (2024 Edition)

作者：Yingyang Xu, Shuang Liu, Xue Wang, Wei Chen, Lei Cheng, Yinshi Guo, J Li, Fang Liu, Ruiling Liu, Juan Meng, Yuemei Sun, Siqin Wang, Qingyu Wei, Yongmei Yu, Huanping Zhang, Zuotao Zhao, Huadong Zhu, Rongfei Zhu, Yuxiang Zhi · 发表于：International Archives of Allergy and Immunology · 年份：2025 · DOI：10.1159/000545808 · 被引用次数：4 · 研究领域：Coagulation, Bradykinin, Polyphosphates, and Angioedema、Hemophilia Treatment and Research、Urticaria and Related Conditions

BACKGROUND: Hereditary angioedema (HAE) is a rare, life-threatening autosomal dominant disorder characterized by recurrent episodes of subcutaneous and submucosal edema. Recent years have witnessed significant advancements in HAE management globally as well as in China, including improved understanding of its pathophysiology and the development of targeted therapies. In China, since the publication of the first national consensus in 2019, accumulating clinical experience and the availability of novel therapeutic agents have created an urgent need to update diagnostic and treatment guidelines to reflect current best practices. SUMMARY: This updated 2024 consensus was developed through collaboration among multidisciplinary experts in allergy, otorhinolaryngology, gastroenterology, dermatology, and emergency medicine across China. It provides comprehensive, evidence-based recommendations for HAE-C1-INH management. This consensus refined diagnostic algorithms incorporating clinical presentation, quantitative/functional C1-INH assays, and complement C4 testing, with genetic sequencing reserved for cases with strong clinical suspicion but normal C1-INH levels/function. It stratified treatment approaches reflecting China's current therapeutic landscape: (1) on-demand therapy with icatibant, which is the only currently approved bradykinin B2 receptor antagonist in China; (2) short-term prophylaxis using androgens or fresh frozen plasma for procedural triggers; (3) long-term prophylax...