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

• Published in: Clinical and experimental immunology Vol. 209; no. 3; pp. 247 - 258
• Main 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, Seneviratne, Suranjith, Shrimpton, Anna, Stroud, Catherine, Thomas, Moira, Townsend, Katie, Vaitla, Prashantha, Verma, Nisha, Williams, Anthony, Burns, Siobhan O, Savic, Sinisa, Richter, Alex G
• Format: Journal Article
• Language: English
• Published: England 29.09.2022
• Subjects: Antibodies, Monoclonal, Humanized; Antibodies, Neutralizing; Antibodies, Viral; COVID-19 - therapy; COVID-19 Drug Treatment; COVID-19 Serotherapy; Dexamethasone; Drug Combinations; Humans; Immunization, Passive; Immunologic Deficiency Syndromes; SARS-CoV-2; United Kingdom - epidemiology; United Kingdom; COVID-19; SARS-CoV-2; secondary immunodeficiencies; primary immunodeficiencies; inborn errors of immunity; hypogammaglobulinemia; lymphopenia
• ISSN: 0009-9104; 1365-2249; 1365-2249
• DOI: 10.1093/cei/uxac008

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.