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Lookup NU author(s): Dr Catherine Stroud
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2023 The AuthorsBackground: Detailed demographic data on people with hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the United Kingdom are relatively limited. Better demographic data would be beneficial in planning service provision, identifying areas of improvement, and improving care. Objective: To obtain more accurate data on the demographics of HAE and acquired C1 inhibitor deficiency in the United Kingdom, including treatment modalities and services available to patients. Methods: A survey was distributed to all centers in the United Kingdom that look after patients with HAE and acquired C1 inhibitor deficiency to collect these data. Results: The survey identified 1152 patients with HAE-1/2 (58% female and 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided by 37 centers across the United Kingdom. This gives a minimum prevalence of 1:59,000 for HAE-1/2 and 1:734,000 for acquired C1 inhibitor deficiency in the United Kingdom. A total of 45% of patients with HAE were on long-term prophylaxis (LTP) with the most used medication being danazol (55% of all patients on LTP). Eighty-two percent of patients with HAE had a home supply of acute treatment with C1 inhibitor or icatibant. A total of 45% of patients had a supply of icatibant and 56% had a supply of C1 inhibitor at home. Conclusions: Data obtained from the survey provide useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the United Kingdom. These data are useful for planning service provision and improving services for these patients.
Author(s): Yong PFK, Coulter T, El-Shanawany T, Garcez T, Hackett S, Jain R, Kiani-Alikhan S, Manson A, Noorani S, Stroud C, Symons C, Sargur R, Steele C, Alachkar H, Anantharachagan A, Arkwright PD, Bernatoniene J, Bhole M, Brown L, Buckland M, Burns S, Chopra C, Darroch J, Drewe E, Edmonds J, Ekbote A, Elkhalifa S, Goddard S, Grosse-Kreul D, Gurugama P, Hague R, Herriot R, Herwadkar A, Hughes SM, Jones L, Lear S, McDermott E, Kham Murng SH, Price A, Redenbaugh V, Richter A, Riordan A, Shackley F, Stichbury J, Springett D, Tarzi MD, Thomas M, Vijayadurai P, Worth A
Publication type: Article
Publication status: Published
Journal: Journal of Allergy and Clinical Immunology: In Practice
Year: 2023
Volume: 11
Issue: 8
Pages: 2476-2483
Print publication date: 01/08/2023
Online publication date: 03/05/2023
Acceptance date: 14/04/2023
Date deposited: 12/06/2023
ISSN (print): 2213-2198
ISSN (electronic): 2213-2201
Publisher: American Academy of Allergy, Asthma and Immunology
URL: https://doi.org/10.1016/j.jaip.2023.04.035
DOI: 10.1016/j.jaip.2023.04.035
PubMed id: 37146882
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