1. Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK
- Author
-
Katie Townsend, Evon Boules, Rachael O’Brien, Sai Murng, Smita Y. Patel, Helen Baxendale, Siraj A. Misbah, Ariharan Anantharachagan, Aarnoud Huissoon, Richard Herriot, Andrew R. Gennery, Kenneth F Baker, David M. Lowe, Lucy Leeman, Suzanne Elcombe, Alex G. Richter, Grant Hayman, Philip D Bright, Moira Thomas, Sarah Goddard, Magdalena Dziadzio, Prashantha M. Vaitla, Sameer Bahal, Betsy Cleave, Dylan James Mac Lochlainn, Elizabeth McDermott, Malini Bhole, Anna Shrimpton, Sujoy Khan, Nisha Verma, William H. Bermingham, Sinisa Savic, Anthony Williams, Gururaj Arumugakani, Lavanya Diwakar, Elizabeth Drewe, James Laffan, Lucy Cliffe, Catherine Stroud, Stephen Jolles, A Herwadkar, Siobhan O. Burns, Adrian M Shields, Shanti Mahabir, Scott Hackett, Sofia Grigoriadou, Sarah Johnston, John Dempster, Hadeil Morsi, Peter Lane, Sadia Noorani, Arthur Price, Tasneem Rahman, Fatima Dhalla, Christopher J A Duncan, Lisa Devlin, Harichandrana Ghanta, Fiona Moghaddas, Charu Chopra, Suranjith Senevirantne, Shuayb Elkhalifa, and Rashmi Jain
- Subjects
medicine.medical_specialty ,inborn errors of immunity ,hypogammaglobulinemia ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,Immunology ,AcademicSubjects/MED00730 ,secondary immunodeficiencies ,Antibodies, Monoclonal, Humanized ,Antibodies, Viral ,Dexamethasone ,Secondary immunodeficiency ,AcademicSubjects/MED00160 ,Internal medicine ,Medicine ,Immunology and Allergy ,Humans ,In patient ,COVID-19 Serotherapy ,business.industry ,SARS-CoV-2 ,Immunization, Passive ,Immunologic Deficiency Syndromes ,COVID-19 ,lymphopenia ,Antibodies, Neutralizing ,United Kingdom ,COVID-19 Drug Treatment ,Drug Combinations ,business ,AcademicSubjects/MED00010 ,AcademicSubjects/MED00690 ,Research Article ,primary immunodeficiencies - 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.
- Published
- 2023