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

Patrick F. K. 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 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

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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 language	English
Pages (from-to)	2476-2483
Number of pages	8
Journal	The Journal of Allergy and Clinical Immunology: In Practice
Volume	11
Issue number	8
Early online date	3 May 2023
DOIs	https://doi.org/10.1016/j.jaip.2023.04.035
Publication status	Published - 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

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10.1016/j.jaip.2023.04.035Licence: CC BY

Final published versionFinal published version, 329 KBLicence: CC BY

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Yong, P. F. K., 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, 11(8), 2476-2483. https://doi.org/10.1016/j.jaip.2023.04.035

@article{e1cf1bd6f66c44b1a18a7d5513044489,

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

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.",

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",

author = "Yong, {Patrick F. K.} 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",

note = "Funding Information: This study was supported by an unrestricted grant from Pharming, which covered editorial services and support for data collection and analysis provided by Neil McCallum-Deighton (Coherent Communications Limited). Copyright: {\textcopyright} 2023. Published by Elsevier Inc.",

year = "2023",

month = aug,

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

language = "English",

volume = "11",

pages = "2476--2483",

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

issn = "2213-2198",

publisher = "American Academy of Allergy, Asthma and Immunology",

number = "8",

}

Yong, PFK, 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, vol. 11, no. 8, pp. 2476-2483. 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 F. K.

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

N1 - Funding Information: This study was supported by an unrestricted grant from Pharming, which covered editorial services and support for data collection and analysis provided by Neil McCallum-Deighton (Coherent Communications Limited). Copyright: © 2023. Published by Elsevier Inc.

PY - 2023/8

Y1 - 2023/8

N2 - 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.

AB - 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.

KW - Hereditary angioedema

KW - acquired C1 inhibitor deficiency

KW - demographics

KW - epidemiology

KW - C1 inhibitor

KW - androgens

KW - icatibant

KW - tranexamic acid

KW - Epidemiology

KW - Acquired C1 inhibitor deficiency

KW - Androgens

KW - Demographics

KW - Tranexamic acid

KW - Icatibant

UR - http://www.scopus.com/inward/record.url?scp=85160858299&partnerID=8YFLogxK

U2 - 10.1016/j.jaip.2023.04.035

DO - 10.1016/j.jaip.2023.04.035

M3 - Article

C2 - 37146882

SN - 2213-2198

VL - 11

SP - 2476

EP - 2483

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

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

IS - 8

ER -

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

Abstract

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