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.
【저자키워드】 COVID-19, SARS-CoV-2, lymphopenia, inborn errors of immunity, Hypogammaglobulinemia, Primary immunodeficiencies, secondary immunodeficiencies., 【초록키워드】 Dexamethasone, Treatment, convalescent plasma, Mortality, Tocilizumab, SARS-COV-2 infection, hospital, Infection, Remdesivir, outcome, risk factor, anti-SARS-CoV-2, COVID-19 treatment, Lymphocyte count, Cohort, therapeutic, Patient, co-morbidity, access, age, network, General population, United Kingdom, monotherapy, CVID, REGN-CoV2, Combination, IFR, high risk, overall mortality, individual, Fatality rate, died, reported, receiving, demonstrated, Increasing, baseline, increased the risk, PID, 【제목키워드】 SARS-COV-2 infection, immunodeficiency, Patient,