Tomas Milota, Marta Sobotkova, Jitka Smetanova, Marketa Bloomfield, Jana Vydlakova, Zita Chovancova, Jiri Litzman, Roman Hakl, Jiri Novak, Ivana Malkusova, Jana Hanzlikova, Dalibor Jilek, Beata Hutyrova, Vitezslav Novak, Irena Krcmova, Anna Sediva and Pavlina Kralickova. Frontiers in Immunology. 2022 February 28. doi.org/10.3389/fimmu.2022.835770 IF: 8,786
MUDr. Tomáš Milota, Ph.D., Ústav imunologie 2. LF UK a FN Motol
Despite the progress in the understanding how COVID-19 infection may impact immunocompromised patients, the data on inborn errors of immunity (IEI) remain limited and ambiguous. Therefore, we examined the risk of severe infection course and hospital admission in a large cohort of patients with IEI. In this multicenter nationwide retrospective survey-based trial, the demographic, clinical, and laboratory data were collected by investigating physicians from 8 national referral centers for the diagnosis and treatment of IEI using a COVID-19-IEI clinical questionnaire. In total, 81 patients with IEI (including 16 with hereditary angioedema, HAE) and confirmed SARS-CoV-2 infection were enrolled, and were found to have a 2.3-times increased (95%CI: 1.44–3.53) risk ratio for hospital admission and a higher mortality ratio (2.4% vs. 1.7% in the general population). COVID-19 severity was associated with the presence of clinically relevant comorbidities, lymphopenia, and hypogammaglobulinemia, but not with age or BMI. No individuals with HAE developed severe disease, despite a hypothesized increased risk due to perturbed bradykinin metabolism. We also demonstrated a high seroconversion rate in antibody-deficient patients and the safety of anti-spike SARS CoV-2 monoclonal antibodies and convalescent plasma. Thus, IEI except for HAE, represent significant risk factors for a severe COVID-19. Therefore, apart from general risk factors, immune system dysregulation may also be involved in the poor outcomes of COVID-19. Despite the study limitations, our results support the findings from previously published trials.