Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2025 Sep 1;46(5):398-405.
doi: 10.2500/aap.2025.46.250048.

Quality of life and burden of disease in patients with hereditary angioedema and their caregivers

Donald S Levy  1 , Fernanda I Nagase  2 , Antoinette Cheung  2 , Deirdre A Rodeberg  3 , Michael E Manning  4
Affiliations
Review

Quality of life and burden of disease in patients with hereditary angioedema and their caregivers

Donald S Levy et al. Allergy Asthma Proc. .

Abstract

Background: Hereditary angioedema (HAE) substantially impairs patients' quality of life (QoL), both physically and psychologically, with unpredictable attacks that cause disruptions in education, work, and social life. Objective: To identify key themes and existing knowledge gaps around the multifaceted burden of HAE. Methods: A literature review was conducted in January 2024 through a search of medical literature data bases. English-language studies considered relevant to patient burden and QoL were selected for analysis. Results: A total of 48 studies were included in the analysis; 50% were cross-sectional and 54% were conducted in North America. Twenty-three studies reported outcomes on QoL and pain, 10 studies reported outcomes on psychological distress, 16 studies reported outcomes on experiences with long-term prophylaxis, 36 studies reported outcomes on HAE attacks, and one study detailed caregiver burden. Patients with HAE had worse QoL compared with the general population, and worse QoL was associated with a higher frequency or severity of attacks, anxiety, and depression. The use of long-term prophylaxis improved QoL, and treatment satisfaction was driven by improvements in mental health and fostering a sense of control and independence. Conclusion: HAE continues to substantially impact QoL of patients. Although recent work has demonstrated progress in standardizing assessment tools for QoL in HAE, additional research is needed to determine the correlation between individual patient factors and QoL.

PubMed Disclaimer

Conflict of interest statement

D.S. Levy has served as a speaker, consultant, and/or researcher for BioCryst, CSL Behring, Cycle Pharma, Kalvista, Pharvaris, and Takeda. F.I. Nagase and A. Cheung are employees of Broadstreet Health Economics and Outcomes Research, which received funding from Astria Therapeutics for this work. D.A. Rodeberg is a paid employee of MedThink SciCom, which was contracted by Astria Therapeutics for this work. M.E. Manning is a speaker, consultant, and/or has received research funding from Astria, BioCryst, BioMarin, CSL Behring, Cycle Pharma, Intellia, Ionis, KalVista, Pharming, Pharvaris, and Takeda

Figures

Figure 1.
Figure 1.
Identified and included studies. *Abstracts were eligible for full-text analysis if they (1) outlined studies conducted in North America or Europe, (2) included adults with hereditary angioedema (HAE) types 1 and/or 2, and (3) presented data on quality of life and impact on daily life, including pain, psychological distress, frequency and severity of attacks, burden and preferences, and/or satisfaction associated with long-term prophylactic treatments, or caregiver burden and quality of life. **“Other” included commentaries, study protocols, or articles in languages other than English.
Figure 2.
Figure 2.
Overall description of included studies. HAE, hereditary angioedema. Adapted from Ref. .
Figure 3.
Figure 3.
QoL assessment tools. QoL = Quality of life; HAE = hereditary angioedema. aIncluded the Angioedema Quality of Life (AE-QoL) and Hereditary Angioedema Quality of Life (HAE-QoL) questionnaires. bIncluded the RAND 36-Item Short Form Health Survey (SF-36) and the EuroQoL 5-Dimension 5-Level (EQ-5D-5L) instrument. cThe Dermatology Life Quality Index (DLQI) was utilized. Adapted from Ref. .
See this image and copyright information in PMC

References

    1. Longhurst HJ, Bork K. Hereditary angioedema: an update on causes, manifestations and treatment. Br J Hosp Med (Lond). 2019; 80:391–398. - PubMed
    1. Busse PJ, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2021; 9:132–150.e3. - PubMed
    1. Bork K, Meng G, Staubach P, et al. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006; 119:267–274. - PubMed
    1. Bygum A, Busse P, Caballero T, et al. Disease severity, activity, impact, and control and how to assess them in patients with hereditary angioedema. Front Med (Lausanne). 2017; 4:212. - PMC - PubMed
    1. Chong-Neto HJ. A narrative review of recent literature of the quality of life in hereditary angioedema patients. World Allergy Organ J. 2023; 16:100758. - PMC - PubMed

LinkOut - more resources