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

{"title":"Worldwide Prevalence of Hereditary Angioedema: A Systematic Review and Meta-Analysis.","authors":"Samuel A Fisch, Andrew G Rundle, Alfred I Neugut, Daniel E Freedberg","doi":"10.1159/000543321","DOIUrl":null,"url":null,"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.","PeriodicalId":13652,"journal":{"name":"International Archives of Allergy and Immunology","volume":" ","pages":"1-9"},"PeriodicalIF":2.5000,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Archives of Allergy and Immunology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1159/000543321","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"ALLERGY","Score":null,"Total":0}

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 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.

查看原文本刊更多论文

遗传性血管性水肿的全球患病率：系统回顾和荟萃分析。

遗传性血管性水肿（HAE）是由C1酯酶抑制剂（C1- inh）蛋白功能障碍或缺乏引起的一种罕见疾病。HAE的真实患病率，以及这种患病率在不同地区之间是否存在差异，尚不确定。方法：为了估计全球HAE的患病率，进行了系统回顾和荟萃分析。采用随机效应模型计算HAE的总患病率，并评估各研究的异质性。结果：2000年至2024年的25项研究纳入了分析，描述了11245例HAE病例。HAE的总患病率为每10万人1.22例（95%可信区间（CI）： 0.91, 1.53），与欧洲和北美相比，亚洲和非洲的患病率较低。1型HAE占大多数病例，女性略占优势。结论：HAE是一种罕见的疾病，全世界每10万人中有1-2例患者。对HAE患病率的真实估计将为该病的护理提供信息，特别是在出现新的治疗方案时。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

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.