Worldwide Prevalence of Hereditary Angioedema: A Systematic Review and Meta-Analysis

Introduction: Hereditary angioedema (HAE) is a rare disease caused by dysfunction or lack of the C1 esterase inhibitor (C1-INH) protein. The true prevalence of HAE, and whether this prevalence differs across regions, is uncertain. Methods: To estimate the prevalence of HAE worldwide, a systematic review and meta-analysis was performed. The pooled prevalence of HAE was calculated using a random-effects model, and heterogeneity across studies was assessed. Results: Twenty-five studies from 2000 to 2024 were included in the analysis, describing 11,245 cases of HAE. The pooled prevalence of HAE was 1.22 (95% confidence interval (CI): 0.91, 1.53) per 100,000, with lower prevalence reported in Asia and Africa compared to Europe and North America. HAE type 1 made up most of the cases, with a slight female predominance. Conclusion: HAE is a rare condition which affects 1-2 individuals per 100,000 population worldwide. A true estimate for the prevalence of HAE will inform care for the condition, especially as new treatment options become available.