Hereditary angioedema (HAE) exacts a significant burden among Dutch patients across different surveyed areas, such as quality of life and treatment satisfaction, according to a study published in The Journal of Allergy and Clinical Immunology: In Practice. 

The recurrent attacks that are characteristic of HAE can range from being symptomatically painful to potentially life-threatening. The frequency and severity of these attacks can differ significantly even among patients of the same genotype. Less severe attacks are often self-resolving, while severe attacks may require hospital care. 

Because HAE is incurable, the main treatment goal is to achieve control of the disease and thus help patients regain some semblance of normalcy in life. Current treatment strategies include both prophylactic care and the acute treatment of attacks. 

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The impact of HAE on patients’ health-related quality of life remains largely unexplored. The authors of this study sought to conduct a cross-sectional survey to assess disease burden in terms of symptomatic control, treatment satisfaction, healthcare/productivity costs, and perception of disease impact on overall quality of life. 

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The research team invited patients with HAE and C1-inhibitor deficiency who were registered with an Amsterdam national reference center to participate in a survey. Eighty-eight adult patients were recruited between July and November 2021, with 69 completing all relevant tasks. Participants were asked to complete the Angioedema Activity Score prospectively for 4 weeks, as well as 1 generic health-related quality of life score (EQ-5D-5L) on an attack-free day and another during an attack day. After the initial 4 weeks, participants were asked to complete a detailed survey on how HAE has impacted various areas of their lives. 

This survey revealed that roughly one-third of participants do not have well-controlled HAE. Healthcare costs for Dutch patients with HAE is around €22,764 ($24,678 USD) per year, mostly spent on medications. The survey also indicated that a substantial segment of this patient population does not meet the main treatment goal of symptomatic control and preservation of quality of life. 

“For this reason, additional prophylactic strategies appear to be needed to improve disease control in a large subset of HAE patients,” the authors of the study concluded. 


Fijen LM, Klein PCG, Cohn DM, Kanters TA. The disease burden and societal costs of hereditary angioedemaJ Allergy Clin Immunol Pract. Published online March 27, 2023. doi:10.1016/j.jaip.2023.03.032