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

IF 2.5 4区医学 Q3 ALLERGY

International Archives of Allergy and Immunology Pub Date : 2025-01-17 DOI:10.1159/000543321

Samuel A Fisch, Andrew G Rundle, Alfred I Neugut, Daniel E Freedberg

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引用次数: 0

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 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.

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来源期刊

International Archives of Allergy and Immunology 医学-过敏

CiteScore

5.60

自引率

3.60%

发文量

105

审稿时长

2 months

期刊介绍： ''International Archives of Allergy and Immunology'' provides a forum for basic and clinical research in modern molecular and cellular allergology and immunology. Appearing monthly, the journal publishes original work in the fields of allergy, immunopathology, immunogenetics, immunopharmacology, immunoendocrinology, tumor immunology, mucosal immunity, transplantation and immunology of infectious and connective tissue diseases.