Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (HAE-C1INH) had high hospitalizations, unnecessary abdominal surgeries, and intubations, contributing to increased mortality, according to a study published in Annals of Allergy, Asthma & Immunology. 

Epidemiological data and information on patient-reported outcomes can influence big-picture decision-making. The United States Hereditary Angioedema Association (US HAEA) have thus collaborated with its Medical Advisory Board to develop a comprehensive HAE registry and biobank, allowing researchers to track the prevalence of HAE-C1INH and evaluate the outcomes of care practices. In addition to holding patient information, the registry also collects responses from patients about their treatment experience through 5 questionnaires. 

The authors of the study sought to determine demographic and epidemiological characteristics of patients with HAE-C1INH and track markers of HAE severity and comorbidities by assessing data from the registry between 2009 and April 7, 2021. They compared HAE-C1INH demographic and epidemiological data with patients who had either HAE and normal C1-inhibitor (HAE-n1-C1INH) or idiopathic nonhistaminergic angioedema (INHA). They then compared the prevalence of comorbidities in the HAE-C1INH cohort with that of the general US population. 

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A total of 596 candidates was identified: 485 had type 1 HAE-C1INH, 96 had angioedema nonmast cell mediated with normal C1-INH (ANM-nl-C1INH; inclusive of 26 HAE-nl-C1INH and 70 INHA patients), and 15 had acquired C1-inhibitor deficiency. The researchers narrowed their focus to patients with HAE-C1INH, HAE-nl-C1INH, or INHA only.

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In each disease category, females outnumbered males (74.4% vs 25.6% in HAE-C1INH, 88.5% vs 11.5% in HAE-nl-C1INH, and 87.1% vs 12.9% in INHA). The primary specialty that made the initial HAE diagnosis and currently manages the patient is overwhelmingly allergy and immunology (63.0% in HAE-C1INH, 80.8% in HAE-nl-C1INH, and 85.5% in INHA). 

Researchers also reported that patients with HAE-C1INH were more likely to suffer from depression and sleep issues, as well as kidney disease, anemia, hepatitis, and angina compared with the general US population. In addition, researchers noted high hospitalizations, intubations, and unnecessary abdominal surgeries among patients with HAE-C1INH and ANM-nl-C1INH. Approximately 36.9% of patients with HAE-C1INH and 15.4% of patients with HAE-nl-C1INH who had a positive family history of HAE died from an attack. 

“In summary, the US HAEA Scientific Registry is a robust tool to learn about the natural history of HAE as well as potential comorbidities that affected patients may experience,” the authors wrote. 


Christiansen SC, Wilmot J, Castaldo AJ, Zuraw BL; US HAEA Medical Advisory Board members. The US HAEA Scientific Registry: hereditary angioedema demographics, disease severity, and comorbiditiesAnn Allergy Asthma Immunol. Published online August 22, 2023. doi:10.1016/j.anai.2023.08.012