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

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 r...

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Main Authors: 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
Format: Journal article
Language:English
Published: Oxford University Press 2022
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Summary: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.