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

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.

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

Summary of studies from 2016 to 2021 reporting on HAE treatment patterns in specific countries.