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

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 prophylaxis with lanadelumab which is the first-line monoclonal anti-kallikrein antibody available in China. Special considerations for pediatric, pregnant, and breast-feeding patients are also addressed.

Key message: As the first updated consensus since 2019, this guideline standardizes HAE management across China while addressing regional disparities in diagnostic capabilities and treatment accessibility. It emphasizes early diagnosis to prevent life-threatening laryngeal edema and promotes individualized treatment strategies tailored to China's therapeutic landscape. Future directions include emerging targeted therapies and the development of biomarkers for disease severity prediction. Implementation of these recommendations is expected to significantly reduce diagnostic delays, improve patient outcomes, and enhance quality of life for individuals with HAE in China.