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. 2022 Sep 29;209(3):247-258.
doi: 10.1093/cei/uxac008.

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

Adrian M Shields  1 , Ariharan Anantharachagan  2 , Gururaj Arumugakani  3 , Kenneth Baker  4 , Sameer Bahal  5 , Helen Baxendale  6 , William Bermingham  7 , Malini Bhole  8 , Evon Boules  9 , Philip Bright  10 , Charu Chopra  11 , Lucy Cliffe  12 , Betsy Cleave  12 , John Dempster  13 , Lisa Devlin  14 , Fatima Dhalla  15 , Lavanya Diwakar  16 , Elizabeth Drewe  12 , Christopher Duncan  17 , Magdalena Dziadzio  18 , Suzanne Elcombe  19 , Shuayb Elkhalifa  20 , Andrew Gennery  21 , Harichandrana Ghanta  22 , Sarah Goddard  16 , Sofia Grigoriadou  23 , Scott Hackett  24 , Grant Hayman  25 , Richard Herriot  26 , Archana Herwadkar  20 , Aarnoud Huissoon  7 , Rashmi Jain  15 , Stephen Jolles  27 , Sarah Johnston  10 , Sujoy Khan  28 , James Laffan  25 , Peter Lane  1 , Lucy Leeman  29 , David M Lowe  30   5 , Shanti Mahabir  31 , Dylan James Mac Lochlainn  15 , Elizabeth McDermott  12 , Siraj Misbah  15 , Fiona Moghaddas  10 , Hadeil Morsi  15 , Sai Murng  25 , Sadia Noorani  32 , Rachael O'Brien  33 , Smita Patel  15 , Arthur Price  31 , Tasneem Rahman  25 , Suranjith Seneviratne  30 , Anna Shrimpton  9 , Catherine Stroud  19 , Moira Thomas  34 , Katie Townsend  25 , Prashantha Vaitla  12 , Nisha Verma  30 , Anthony Williams  22 , Siobhan O Burns  30   5 , Sinisa Savic  3 , Alex G Richter  1
Affiliations

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

Adrian M Shields et al. Clin Exp Immunol. .

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: COVID-19; SARS-CoV-2; hypogammaglobulinemia; inborn errors of immunity; lymphopenia; primary immunodeficiencies; secondary immunodeficiencies.

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Figures

Graphical Abstract
Graphical Abstract
Figure 1:
Figure 1:
Risk factors for mortality from COVID-19 in patients with PID and SID. Age, baseline lymphocyte count, and body mass index are compared between COVID-19 survivors and non-survivors in patients with (A) PID on immunoglobulin replacement, (B) Common variable immunodeficiency, and (C) Secondary immunodeficiency. Statistical comparison of the groups is made using the 2-tailed Mann–Whitney U test. ∗P < 0.0001, ∗∗P = 0.003, ∗∗∗P = 0.03. Red shaded area denotes lymphopenia (<1.0 × 109/L).
See this image and copyright information in PMC

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