Prevalence and Incidence of Hereditary Angioedema: A Systematic Literature Review.

Authors

Timothy J Craig, Marc A Riedl, Tukisa D Smith, Ellen Kasireddy, Otto L T Lam, Monica R McClain

Published in

International archives of allergy and immunology. Pages 1-25. Jul 22, 2026. Epub Jul 22, 2026.

Abstract

Prior reports on the global prevalence of hereditary angioedema (HAE) have varied widely, and it remains uncertain as to what extent such variation can be attributed to regional differences. More accurate estimates of HAE prevalence can potentially raise awareness among clinicians and reduce diagnostic delay. This systematic literature review identified studies reporting on HAE prevalence, with the aim of generating pooled global/regional estimates.
Relevant studies were identified through a systematic search of MEDLINE® and Embase (inception to June 21, 2024). Study selection, data extraction, and quality assessment were performed in duplicate by two independent reviewers; 5,225 studies were screened, with 41 publications (pertaining to 40 unique studies) being included.
Most studies were conducted in Europe (n = 22); other regions included Northern Eurasia (n = 1), North (n = 3) and South America (n =1), Africa (n= 1), Middle East (n = 1), East Asia (n = 6), and Oceania (n = 1). Diagnostic methodology varied greatly, with a minority of studies citing an established guideline. Pooled diagnosed annual prevalence estimates (95% confidence interval) were 1.31 per 100,000 (0.83-2.08; type I HAE), 0.20 per 100,000 (0.14-0.29; type II HAE), 0.06 per 100,000 (0.01-0.40; HAE with normal C1-inhibitor [INH]), and 1.70 per 100,000 (1.58-1.83; combined types). Pooled estimates by region were 1.43 per 100,000 (0.99-2.08) and 1.64 per 100,000 (1.02-2.64) in North America and Europe, respectively, which were higher than individual estimates from the other regions.
This review determined pooled, minimal diagnosed annual prevalence estimates for type I HAE, type II HAE, HAE-nC1-INH, and type I/II/nC1-INH combined. The pooled estimate for European countries was relatively higher than that for North American countries, as well as individually reported estimates in other regions. Nevertheless, valid comparisons across countries were hindered by a large degree of heterogeneity in data sources and diagnostic methodology. Additional, high-quality studies using standardized diagnostic methods are required to elucidate whether regional differences can be truly attributed to ethnic differences in phenotypic and genotypic variability.

PMID:
42485266
Bibliographic data and abstract were imported from PubMed on 23 Jul 2026.

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