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Review
. 2022 Aug;42(6):1293-1299.
doi: 10.1007/s10875-022-01274-w. Epub 2022 May 23.

Inborn Errors of Immunity on the Island of Ireland - a Cross-Jurisdictional UKPID/ESID Registry Report

Paul Ryan  1 , Vyanka Redenbaugh  2 , Jayne McGucken  2 , Gerhard Kindle  3 , Lisa A Devlin  2 , Tanya Coulter  2 , Matthew S Buckland  4 , Mikko R J Seppänen  5 , Niall P Conlon  6 , Conleth Feighery  6 , J David M Edgar  6
Affiliations
Review

Inborn Errors of Immunity on the Island of Ireland - a Cross-Jurisdictional UKPID/ESID Registry Report

Paul Ryan et al. J Clin Immunol. 2022 Aug.

Erratum in

Abstract

The epidemiology of inborn errors of immunity (IEI) in the Republic of Ireland was first published in 2005 but has not been updated since. IEI prevalence data from Northern Ireland was last published in 2018. Using data from the United Kingdom Primary Immune Deficiency (UKPID) and European Society for Immunodeficiencies (ESID) registries, we reviewed all registered cases of IEI affecting adult patients ≥ 18 years of age from the two largest immunology specialist centres in Northern Ireland and the Republic of Ireland, respectively and calculated the combined minimum adult prevalence of IEI on the island of Ireland for the first time. We also recorded data pertaining to presenting symptoms of IEI, diagnostic delay, immunoglobulin data, and genetic testing, as well as briefly reporting data pertaining to secondary immunodeficiency in both countries. As of 1 May 2020, we identified a minimum adult IEI prevalence in Ireland of 8.85/100,000 population.

Keywords: ESID; IEI (inborn errors of immunity); Immunodeficiency; UKPID; prevalence; registry.

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Conflict of interest statement

J. David M. Edgar has received speaker fees/lecture fees from Takeda Pharmaceutical Company and ALK Abello and has received advisor fees on the advisory board of Pharming Group. Lisa A. Devlin has received sponsorship to attend ESID from CSL Behring and speaker fees from A/K to speak at a GP conference. Niall Conlon has received an unrestricted grant from Takeda Pharmaceutical Company supporting research in an unrelated project (COVID-19).

All the other authors declare no conflict of interest.

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