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Review
. 2023 May;13(5):e12243.
doi: 10.1002/clt2.12243.

Current challenges and future opportunities in patient-focused management of hereditary angioedema: A narrative review

Anete S Grumach  1 , Noga Gadir  2 , Aharon Kessel  3 , Ashley Yegin  2 , Inmaculada Martinez-Saguer  4 , Jonathan A Bernstein  5
Affiliations
Review

Current challenges and future opportunities in patient-focused management of hereditary angioedema: A narrative review

Anete S Grumach et al. Clin Transl Allergy. 2023 May.

Abstract

Patients with hereditary angioedema (HAE) experience a high burden of disease due to unpredictable, painful, disfiguring, and potentially life-threatening HAE attacks. Multiple HAE-specific medications for the on-demand treatment, short-term and long-term prophylaxis of HAE attacks have entered the market in recent years; however, the availability and access to these medications may vary between different countries. For this review, PubMed and EMBASE databases were searched for guidelines, consensus statements, and other publications on HAE management as well as publications on quality of life in patients with HAE. The current guidelines and recent literature on HAE management in specific countries are summarized with the aim to highlight the similarities and differences between guideline recommendations and the country-specific clinical practice. Improvement in quality of life, which is a key goal in HAE management, is also discussed and the country-specific trends are highlighted. Finally, the ways to achieve a more patient-centric approach to HAE management within the framework set by the clinical management guidelines are examined.

Keywords: C1 inhibitor; guidelines; hereditary angioedema; management; quality of life.

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Conflict of interest statement

ASG has received speaker/consultancy fees from CSL Behring, Multicare, Pharvaris, and Takeda; and a grant of researcher initiative from Takeda.

NG and AY are employees of and hold stock/options in Takeda.

AK has received travel grants from Pharming and Takeda, and honoraria from CSL Behring and Takeda.

IMS has received honoraria, research funding, consultancy fees and travel grants from and/or participated in advisory boards for BioCryst, CSL Behring, Pharming, and Takeda.

JAB has been a clinical investigator for BioCryst, CSL Behring, Ionis, KalVista, Pharming, and Takeda; speaker for BioCryst, CSL Behring, Pharming, and Takeda; consultant for Astria, BioCryst, BioMarin, CSL Behring, Cycle, Ionis, KalVista, ONO, Pharming, Pharvaris, and Takeda; and is an advisory board member of the US Hereditary Angioedema Association.

Figures

FIGURE 1
FIGURE 1
Summary of HAE guidelines and consensus papers published in 2017–2022. AE, angioedema; EAACI, European Academy of Allergy and Clinical Immunology; HAE, hereditary angioedema; HAEA, Hereditary Angioedema Association; QoL, quality of life; WAO, World Allergy Organization.
FIGURE 2
FIGURE 2
Summary of studies from 2016 to 2021 reporting on HAE treatment patterns in specific countries.
See this image and copyright information in PMC

References

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