A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom

Patrick F. K. Yong (Lead / Corresponding author), Tanya Coulter, Tariq El-Shanwany, Tomaz Garcez, Scott Hackett, Rashmi Jain, Sorena Kiani-Alikhan, Ania Manson, Sadia Noorani, Catherine Stroud, Christine Symons, Ravishankar Sargur, Cathal Steele, Hana Alachkar, Ariharan Anantharachagan, Peter D. Arkwright, Jolanta Bernatoniene, Malini Bhole, Lindsay Brown, Matthew BucklandSiobhan Burns, Charu Chopra, James Darroch, Elizabeth Drewe, Jillian Edmonds, Anjali Ekbote, Shuayb Elkhalifa, Sarah Goddard, Dorothea Grosse-Kreul, Padmalal Gurugama, Rosie Hague, Richard Herriot, Archana Herwadkar, Stephen M. Hughes, Laura Jones, Sara Lear, Elizabeth McDermott, Sai Hurng Kham Murng, Arthur Price, Vyanka Redenbaugh, Alex Richter, Andrew Riordan, Fiona Shackley, Julia Stichbury, Debbie Springett, Michael D. Tarzi, Moira Thomas, Pavaladurai Vijayadurai, Austen Worth

    Research output: Contribution to journalArticlepeer-review

    6 Citations (Scopus)
    63 Downloads (Pure)

    Abstract

    Background: 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.

    Original languageEnglish
    Pages (from-to)2476-2483
    Number of pages8
    JournalThe Journal of Allergy and Clinical Immunology: In Practice
    Volume11
    Issue number8
    Early online date3 May 2023
    DOIs
    Publication statusPublished - Aug 2023

    Keywords

    • Hereditary angioedema
    • acquired C1 inhibitor deficiency
    • demographics
    • epidemiology
    • C1 inhibitor
    • androgens
    • icatibant
    • tranexamic acid
    • Epidemiology
    • Acquired C1 inhibitor deficiency
    • Androgens
    • Demographics
    • Tranexamic acid
    • Icatibant

    ASJC Scopus subject areas

    • Immunology and Allergy

    Fingerprint

    Dive into the research topics of 'A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom'. Together they form a unique fingerprint.

    Cite this