Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

Adrian M Shields; Ariharan Anantharachagan; Gururaj Arumugakani; Kenneth Baker; Sameer Bahal; Helen Baxendale; William Bermingham; Malini Bhole; Evon Boules; Philip Bright; Charu Chopra; Lucy Cliffe; Betsy Cleave; John Dempster; Lisa Devlin; Fatima Dhalla; Lavanya Diwakar; Elizabeth Drewe; Christopher Duncan; Magdalena Dziadzio; Suzanne Elcombe; Shuayb Elkhalifa; Andrew Gennery; Harichandrana Ghanta; Sarah Goddard; Sofia Grigoriadou; Scott Hackett; Grant Hayman; Richard Herriot; Archana Herwadkar; Aarnoud Huissoon; Rashmi Jain; Stephen Jolles; Sarah Johnston; Sujoy Khan; James Laffan; Peter Lane; Lucy Leeman; David M Lowe; Shanti Mahabir; Dylan James Mac Lochlainn; Elizabeth McDermott; Siraj Misbah; Fiona Moghaddas; Hadeil Morsi; Sai Murng; Sadia Noorani; Rachael O'Brien; Smita Patel; Arthur Price; Tasneem Rahman; Suranjith Seneviratne; Anna Shrimpton; Catherine Stroud; Moira Thomas; Katie Townsend; Prashantha Vaitla; Nisha Verma; Anthony Williams; Siobhan O Burns; Sinisa Savic; Alex G Richter

doi:10.1093/cei/uxac008

Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

Adrian M Shields, Ariharan Anantharachagan, Gururaj Arumugakani, Kenneth Baker, Sameer Bahal, Helen Baxendale, William Bermingham, Malini Bhole, Evon Boules, Philip Bright, Charu Chopra, Lucy Cliffe, Betsy Cleave, John Dempster, Lisa Devlin, Fatima Dhalla, Lavanya Diwakar, Elizabeth Drewe, Christopher Duncan, Magdalena DziadzioSuzanne Elcombe, Shuayb Elkhalifa, Andrew Gennery, Harichandrana Ghanta, Sarah Goddard, Sofia Grigoriadou, Scott Hackett, Grant Hayman, Richard Herriot, Archana Herwadkar, Aarnoud Huissoon, Rashmi Jain, Stephen Jolles, Sarah Johnston, Sujoy Khan, James Laffan, Peter Lane, Lucy Leeman, David M Lowe, Shanti Mahabir, Dylan James Mac Lochlainn, Elizabeth McDermott, Siraj Misbah, Fiona Moghaddas, Hadeil Morsi, Sai Murng, Sadia Noorani, Rachael O'Brien, Smita Patel, Arthur Price, Tasneem Rahman, Suranjith Seneviratne, Anna Shrimpton, Catherine Stroud, Moira Thomas, Katie Townsend, Prashantha Vaitla, Nisha Verma, Anthony Williams, Siobhan O Burns^*, Sinisa Savic^*, Alex G Richter^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

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Abstract

In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.

Original language	English
Pages (from-to)	247-258
Number of pages	12
Journal	Clinical and Experimental Immunology
Volume	209
Issue number	3
Early online date	28 Jan 2022
DOIs	https://doi.org/10.1093/cei/uxac008
Publication status	Published - Sept 2022

Bibliographical note

Keywords

Antibodies, Monoclonal, Humanized
Antibodies, Neutralizing
Antibodies, Viral
COVID-19/drug therapy
Dexamethasone
Drug Combinations
Humans
Immunization, Passive
Immunologic Deficiency Syndromes
SARS-CoV-2
Sudden Infant Death
United Kingdom/epidemiology
hypogammaglobulinemia
COVID-19
secondary immunodeficiencies
primary immunodeficiencies
inborn errors of immunity
lymphopenia

ASJC Scopus subject areas

Immunology and Allergy
Immunology

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Shields, A. M., Anantharachagan, A., Arumugakani, G., Baker, K., Bahal, S., Baxendale, H., Bermingham, W., Bhole, M., Boules, E., Bright, P., Chopra, C., Cliffe, L., Cleave, B., Dempster, J., Devlin, L., Dhalla, F., Diwakar, L., Drewe, E., Duncan, C., ... Richter, A. G. (2022). Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK. Clinical and Experimental Immunology, 209(3), 247-258. https://doi.org/10.1093/cei/uxac008

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title = "Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK",

abstract = "In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.",

keywords = "Antibodies, Monoclonal, Humanized, Antibodies, Neutralizing, Antibodies, Viral, COVID-19/drug therapy, Dexamethasone, Drug Combinations, Humans, Immunization, Passive, Immunologic Deficiency Syndromes, SARS-CoV-2, Sudden Infant Death, United Kingdom/epidemiology, hypogammaglobulinemia, COVID-19, secondary immunodeficiencies, primary immunodeficiencies, inborn errors of immunity, lymphopenia",

author = "Shields, {Adrian M} and Ariharan Anantharachagan and Gururaj Arumugakani and Kenneth Baker and Sameer Bahal and Helen Baxendale and William Bermingham and Malini Bhole and Evon Boules and Philip Bright and Charu Chopra and Lucy Cliffe and Betsy Cleave and John Dempster and Lisa Devlin and Fatima Dhalla and Lavanya Diwakar and Elizabeth Drewe and Christopher Duncan and Magdalena Dziadzio and Suzanne Elcombe and Shuayb Elkhalifa and Andrew Gennery and Harichandrana Ghanta and Sarah Goddard and Sofia Grigoriadou and Scott Hackett and Grant Hayman and Richard Herriot and Archana Herwadkar and Aarnoud Huissoon and Rashmi Jain and Stephen Jolles and Sarah Johnston and Sujoy Khan and James Laffan and Peter Lane and Lucy Leeman and Lowe, {David M} and Shanti Mahabir and Lochlainn, {Dylan James Mac} and Elizabeth McDermott and Siraj Misbah and Fiona Moghaddas and Hadeil Morsi and Sai Murng and Sadia Noorani and Rachael O'Brien and Smita Patel and Arthur Price and Tasneem Rahman and Suranjith Seneviratne and Anna Shrimpton and Catherine Stroud and Moira Thomas and Katie Townsend and Prashantha Vaitla and Nisha Verma and Anthony Williams and Burns, {Siobhan O} and Sinisa Savic and Richter, {Alex G}",

note = "{\textcopyright} The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Immunology.",

year = "2022",

month = sep,

doi = "10.1093/cei/uxac008",

language = "English",

volume = "209",

pages = "247--258",

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Shields, AM, Anantharachagan, A, Arumugakani, G, Baker, K, Bahal, S, Baxendale, H, Bermingham, W, Bhole, M, Boules, E, Bright, P, Chopra, C, Cliffe, L, Cleave, B, Dempster, J, Devlin, L, Dhalla, F, Diwakar, L, Drewe, E, Duncan, C, Dziadzio, M, Elcombe, S, Elkhalifa, S, Gennery, A, Ghanta, H, Goddard, S, Grigoriadou, S, Hackett, S, Hayman, G, Herriot, R, Herwadkar, A, Huissoon, A, Jain, R, Jolles, S, Johnston, S, Khan, S, Laffan, J, Lane, P, Leeman, L, Lowe, DM, Mahabir, S, Lochlainn, DJM, McDermott, E, Misbah, S, Moghaddas, F, Morsi, H, Murng, S, Noorani, S, O'Brien, R, Patel, S, Price, A, Rahman, T, Seneviratne, S, Shrimpton, A, Stroud, C, Thomas, M, Townsend, K, Vaitla, P, Verma, N, Williams, A, Burns, SO, Savic, S & Richter, AG 2022, 'Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK', Clinical and Experimental Immunology, vol. 209, no. 3, pp. 247-258. https://doi.org/10.1093/cei/uxac008

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T1 - Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

AU - Shields, Adrian M

AU - Anantharachagan, Ariharan

AU - Arumugakani, Gururaj

AU - Baker, Kenneth

AU - Bahal, Sameer

AU - Baxendale, Helen

AU - Bermingham, William

AU - Bhole, Malini

AU - Boules, Evon

AU - Bright, Philip

AU - Chopra, Charu

AU - Cliffe, Lucy

AU - Cleave, Betsy

AU - Dempster, John

AU - Devlin, Lisa

AU - Dhalla, Fatima

AU - Diwakar, Lavanya

AU - Drewe, Elizabeth

AU - Duncan, Christopher

AU - Dziadzio, Magdalena

AU - Elcombe, Suzanne

AU - Elkhalifa, Shuayb

AU - Gennery, Andrew

AU - Ghanta, Harichandrana

AU - Goddard, Sarah

AU - Grigoriadou, Sofia

AU - Hackett, Scott

AU - Hayman, Grant

AU - Herriot, Richard

AU - Herwadkar, Archana

AU - Huissoon, Aarnoud

AU - Jain, Rashmi

AU - Jolles, Stephen

AU - Johnston, Sarah

AU - Khan, Sujoy

AU - Laffan, James

AU - Lane, Peter

AU - Leeman, Lucy

AU - Lowe, David M

AU - Mahabir, Shanti

AU - Lochlainn, Dylan James Mac

AU - McDermott, Elizabeth

AU - Misbah, Siraj

AU - Moghaddas, Fiona

AU - Morsi, Hadeil

AU - Murng, Sai

AU - Noorani, Sadia

AU - O'Brien, Rachael

AU - Patel, Smita

AU - Price, Arthur

AU - Rahman, Tasneem

AU - Seneviratne, Suranjith

AU - Shrimpton, Anna

AU - Stroud, Catherine

AU - Thomas, Moira

AU - Townsend, Katie

AU - Vaitla, Prashantha

AU - Verma, Nisha

AU - Williams, Anthony

AU - Burns, Siobhan O

AU - Savic, Sinisa

AU - Richter, Alex G

PY - 2022/9

Y1 - 2022/9

N2 - In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.

AB - In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID or SID have now been reported in the UK. The overall mortality within the cohort was 17.7% (n = 55/310). Individuals with CVID demonstrated an infection fatality rate (IFR) of 18.3% (n = 17/93), individuals with PID receiving IgRT had an IFR of 16.3% (n = 26/159) and individuals with SID, an IFR of 27.2% (n = 25/92). Individuals with PID and SID had higher inpatient mortality and died at a younger age than the general population. Increasing age, low pre-SARS-CoV-2 infection lymphocyte count and the presence of common co-morbidities increased the risk of mortality in PID. Access to specific COVID-19 treatments in this cohort was limited: only 22.9% (n = 33/144) of patients admitted to the hospital received dexamethasone, remdesivir, an anti-SARS-CoV-2 antibody-based therapeutic (e.g. REGN-COV2 or convalescent plasma) or tocilizumab as a monotherapy or in combination. Dexamethasone, remdesivir, and anti-SARS-CoV-2 antibody-based therapeutics appeared efficacious in PID and SID. Compared to the general population, individuals with PID or SID are at high risk of mortality following SARS-CoV-2 infection. Increasing age, low baseline lymphocyte count, and the presence of co-morbidities are additional risk factors for poor outcome in this cohort.

KW - Antibodies, Monoclonal, Humanized

KW - Antibodies, Neutralizing

KW - Antibodies, Viral

KW - COVID-19/drug therapy

KW - Dexamethasone

KW - Drug Combinations

KW - Humans

KW - Immunization, Passive

KW - Immunologic Deficiency Syndromes

KW - SARS-CoV-2

KW - Sudden Infant Death

KW - United Kingdom/epidemiology

KW - hypogammaglobulinemia

KW - COVID-19

KW - secondary immunodeficiencies

KW - primary immunodeficiencies

KW - inborn errors of immunity

KW - lymphopenia

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U2 - 10.1093/cei/uxac008

DO - 10.1093/cei/uxac008

M3 - Article

C2 - 35641155

SN - 0009-9104

VL - 209

SP - 247

EP - 258

JO - Clinical and Experimental Immunology

JF - Clinical and Experimental Immunology

IS - 3

ER -

Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

Abstract

Bibliographical note

Keywords

ASJC Scopus subject areas

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