The US Hereditary Angioedema Association Scientific Registry: hereditary angioedema demographics, disease severity, and comorbidities.

Autor:	Christiansen SC; Department of Medicine, University of California San Diego, La Jolla, California., Wilmot J; US Hereditary Angioedema Association, Fairfax, Virginia., Castaldo AJ; US Hereditary Angioedema Association, Fairfax, Virginia., Zuraw BL; Department of Medicine, University of California San Diego, La Jolla, California; Medicine Service, Veterans Administration Healthcare, San Diego, California. Electronic address: bzuraw@health.ucsd.edu.
Jazyk:	angličtina
Zdroj:	Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology [Ann Allergy Asthma Immunol] 2023 Dec; Vol. 131 (6), pp. 766-774.e8. Date of Electronic Publication: 2023 Aug 23.
DOI:	10.1016/j.anai.2023.08.012
Abstrakt:	Background: Hereditary angioedema (HAE) and idiopathic nonhistaminergic angioedema (INHA) are ultra-rare diseases whose natural histories and comorbidities are incompletely understood. Objective: To develop a national patient-centric registry to address these deficiencies in our knowledge and improve our ability to assess the real-world impact of therapeutic interventions. Methods: Data from members of the US HAE Association were collected into an online registry between 2009 and April 7, 2021. Cohorts were categorized by reported physician diagnosis. Patient reported data were collected using a series of questionnaires. Demographic, natural history, and family history outcomes of the HAE due to C1 inhibitor deficiency (HAE-C1INH) participants were compared with those of the combined HAE with normal C1 inhibitor (HAE-nl-C1INH) plus INHA group. The prevalence of comorbid conditions in the HAE-C1INH group was compared with the general US population. Results: A total of 485 HAE-C1INH, 26 HAE-nl-C1INH, and 70 INHA participants were included in the analysis. Delay to diagnosis was shorter in HAE-C1INH (5 vs 11 years), but both had decreasing delays over time. Differences in attack frequency and location were found between the groups. Morbidity surrogates including emergency department visits, hospitalizations, unnecessary abdominal surgeries, and intubations were strikingly high as was mortality with 36.9% of HAE-C1INH and 15.4% of HAE-nl-C1INH participants reporting family members who died from a HAE attack. Females with HAE-C1INH had a significant increase in the prevalence of depression, sleep disorders, kidney disease, anemia, and hepatitis. Cardiovascular comorbidities were significantly reduced in the HAE-C1INH group. Conclusion: The US HAEA Scientific Registry provides a mechanism to enhance our knowledge of HAE and INHA. (Published by Elsevier Inc.)
Databáze:	MEDLINE
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