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

Samuel A Fisch¹, Andrew G Rundle², Alfred I Neugut^2,3

¹Department of Epidemiology, Mailman School of Public Health, New York, New York, USA, saf2217@cumc.columbia.edu.

International Archives of Allergy and Immunology

|January 19, 2025

Abstract

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 were performed. The pooled prevalence of HAE was calculated using a random-effects model, and heterogeneity across studies was assessed.

Results:

Twenty-four studies from 2000 to 2024 were included in the analysis, describing 11,245 cases of HAE. The pooled prevalence of HAE was 1.22 cases per 100,000 people (95% confidence interval [CI]: 0.91, 1.53), with lower prevalences 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 people worldwide. A true estimate of the prevalence of HAE will inform care for the condition, especially as new treatment options become available.

Keywords:

Allergy and immunology Angioedema Bradykinin C1 esterase inhibitor Complement Diagnosis Genetics Global prevalence Hereditary angioedema

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