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

Patrick Fk. Yong, 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

15 Downloads (Pure)

Abstract

Background
Detailed demographic data on people with hereditary angioedema and acquired C1 inhibitor deficiency in the UK 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 hereditary angioedema and acquired C1 inhibitor deficiency in the UK, including treatment modalities and services available to patients.

Methods
A survey was distributed to all centres in the UK who look after patients with hereditary angioedema and acquired C1 inhibitor deficiency to collect these data.

Results
The survey identified 1152 patients with HAE-1/2 (58% female, 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided by 37 centres across the UK. This gives a minimum prevalence of 1:59,000 for HAE-1/2, and 1: 734,000 for acquired C1 inhibitor deficiency in the UK. 45% of patients with HAE were on long-term prophylaxis with the most used medication being danazol (55% of all patients on LTP). 82% of patients with HAE had a home supply of acute treatment with C1 inhibitor or icatibant. 45% of patients had a supply of icatibant and 56% had a supply of C1 inhibitor at home.

Conclusions
Data obtained from the survey provides useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the UK. These data are useful for planning service provision and improving services for these patients.
Original languageEnglish
JournalThe Journal of Allergy and Clinical Immunology: In Practice
Early online date3 May 2023
DOIs
Publication statusE-pub ahead of print - 3 May 2023

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