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Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK
Author(s) -
Adrian M. Shields,
Ariharan Anantharachagan,
Gururaj Arumugakani,
Kenneth F. 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,
C.J. Duncan,
Magdalena Dziadzio,
Suzanne Elcombe,
Shuayb Elkhalifa,
Andrew R. 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 J. L. Lane,
Lucy Leeman,
David M. Lowe,
Shanti Mahabir,
Dylan J. Mac Lochlainn,
Elizabeth McDermott,
Siraj Misbah,
Fiona Moghaddas,
Hadeil Morsi,
Sai Murng,
Sadia Noorani,
Rachael O’Brien,
Smita Y. Patel,
Arthur Price,
Tasneem Rahman,
Suranjith L. Seneviratne,
Anna Shrimpton,
Catherine Stroud,
Moira Thomas,
Katie Townsend,
Prashantha Vaitla,
Nisha Verma,
Anthony Williams,
Siobhan O. Burns,
Sinisa Savic,
Alex Richter
Publication year - 2022
Publication title -
clinical and experimental immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.329
H-Index - 135
eISSN - 1365-2249
pISSN - 0009-9104
DOI - 10.1093/cei/uxac008
Subject(s) - covid-19 , medicine , human immunodeficiency virus (hiv) , immunology , primary immunodeficiency , virology , infectious disease (medical specialty) , immune system , disease , outbreak
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.

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