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 Buckland; Siobhan 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

doi:10.1016/j.jaip.2023.04.035

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 journal › Article › peer-review

37 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 language	English
Journal	The Journal of Allergy and Clinical Immunology: In Practice
Early online date	3 May 2023
DOIs	https://doi.org/10.1016/j.jaip.2023.04.035
Publication status	E-pub ahead of print - 3 May 2023

Access to Document

10.1016/j.jaip.2023.04.035

HAE survey JACI-IP revision v2 CLEANAccepted author manuscript, 682 KB

Cite this

Yong, P. F., Coulter, T., El-Shanwany, 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, P. D., Bernatoniene, J., Bhole, M., Brown, L., ... Worth, A. (2023). A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom. The Journal of Allergy and Clinical Immunology: In Practice. Advance online publication. https://doi.org/10.1016/j.jaip.2023.04.035

@article{ff89f816f6de42e891bc4d91d37cc9c4,

title = "A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom",

abstract = "BackgroundDetailed 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.ObjectiveTo 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.MethodsA 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.ResultsThe 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.ConclusionsData 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.",

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

year = "2023",

month = may,

day = "3",

doi = "10.1016/j.jaip.2023.04.035",

language = "English",

journal = "The Journal of Allergy and Clinical Immunology: In Practice",

issn = "2213-2201",

publisher = "Elsevier BV",

}

Yong, PF, Coulter, T, El-Shanwany, 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 2023, 'A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom', The Journal of Allergy and Clinical Immunology: In Practice. https://doi.org/10.1016/j.jaip.2023.04.035

TY - JOUR

T1 - A national survey of hereditary angioedema and acquired C1 inhibitor deficiency in the United Kingdom

AU - Yong, Patrick Fk.

AU - Coulter, Tanya

AU - El-Shanwany, Tariq

AU - Garcez, Tomaz

AU - Hackett, Scott

AU - Jain, Rashmi

AU - Kiani-Alikhan, Sorena

AU - Manson, Ania

AU - Noorani, Sadia

AU - Stroud, Catherine

AU - Symons, Christine

AU - Sargur, Ravishankar

AU - Steele, Cathal

AU - Alachkar, Hana

AU - Anantharachagan, Ariharan

AU - Arkwright, Peter D.

AU - Bernatoniene, Jolanta

AU - Bhole, Malini

AU - Brown, Lindsay

AU - Buckland, Matthew

AU - Burns, Siobhan

AU - Chopra, Charu

AU - Darroch, James

AU - Drewe, Elizabeth

AU - Edmonds, Jillian

AU - Ekbote, Anjali

AU - Elkhalifa, Shuayb

AU - Goddard, Sarah

AU - Grosse-Kreul, Dorothea

AU - Gurugama, Padmalal

AU - Hague, Rosie

AU - Herriot, Richard

AU - Herwadkar, Archana

AU - Hughes, Stephen M.

AU - Jones, Laura

AU - Lear, Sara

AU - Mcdermott, Elizabeth

AU - Kham murng, Sai Hurng

AU - Price, Arthur

AU - Redenbaugh, Vyanka

AU - Richter, Alex

AU - Riordan, Andrew

AU - Shackley, Fiona

AU - Stichbury, Julia

AU - Springett, Debbie

AU - Tarzi, Michael D.

AU - Thomas, Moira

AU - Vijayadurai, Pavaladurai

AU - Worth, Austen

PY - 2023/5/3

Y1 - 2023/5/3

N2 - BackgroundDetailed 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.ObjectiveTo 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.MethodsA 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.ResultsThe 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.ConclusionsData 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.

AB - BackgroundDetailed 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.ObjectiveTo 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.MethodsA 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.ResultsThe 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.ConclusionsData 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.

U2 - 10.1016/j.jaip.2023.04.035

DO - 10.1016/j.jaip.2023.04.035

M3 - Article

SN - 2213-2201

JO - The Journal of Allergy and Clinical Immunology: In Practice

JF - The Journal of Allergy and Clinical Immunology: In Practice

ER -

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

Abstract

Access to Document

Fingerprint

Cite this