Outcomes Following SARS-CoV-2 Infection in Patients With Primary and Secondary Immunodeficiency in The United Kingdom
Authors: Shields Adrian M , Anantharachagan Ariharan , Arumugakani Gururaj , Baker Kenneth , Bahal Sameer , Baxendale Helen , Bermingham William , Bhole Malini , Boules Evon , Bright Philip , Chopra Charu , Cliffe Lucy , Cleave Betsy , Dempster John , Devlin Lisa , Dhalla Fatima , Diwakar Lavanya , Drewe Elizabeth , Duncan Christopher , Dziadzio Magdalena , Elcombe Suzanne , Elkhalifa Shuayb , Gennery Andrew , Ghanta Harichandrana , Goddard Sarah , Grigoriadou Sofia , Hackett Scott , Hayman Grant , Herriot Richard , Herwadkar Archana , Huissoon Aarnoud , Jain Rashmi , Jolles Stephen , Johnston Sarah , Khan Sujoy , Laffan James , Lane Peter , Leeman Lucy , Lowe David M. , Mahabir Shanti , Lochlainn Dylan James Mac , McDermott Elizabeth , Misbah Siraj , Moghaddas Fiona , Morsi Hadeil , Murng Sai , Noorani Sadia , O’Brien Rachael , Patel Smita , Price Arthur , Rahman Tasneem , Senevirantne Suranjith , Shrimpton Anna , Stroud Catherine , Thomas Moira , Townsend Katie , Vaitla Prashantha , Verma Nisha , Williams Anthony , Burns Siobhan O. , Savic Sinisa , Richter Alex G. 
Published: October 2021

Abstract
Purpose
To define the burden of morbidity and mortality arising from COVID-19 in individuals with primary (PID) and secondary immunodeficiency (SID) in the United Kingdom.
Methods
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. Anonymised demographic data, pre-SARS-CoV-2 infection lymphocyte counts, co-morbidities, targeted treatments and outcomes were collected. Three groups were analysed in further detail: individuals with common variable immunodeficiency (CVID), individuals with any PID, including CVID, receiving immunoglobulin replacement therapy (IgRT) and individuals with secondary immunodeficiency.
Results
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 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.
Conclusion
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.