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.

Center for Clinical Immunology Hospital Ceske Budejovice Ceske Budejovice Czechia

Department of Allergology and Clinical Immunology Faculty of Medicine Masaryk University and St Anne's University Hospital in Brno Brno Czechia

Department of Allergology and Clinical Immunology Institute of Health in Usti nad Labem Usti nad Labem Czechia

Department of Allergology and Clinical Immunology University Hospital in Olomouc Olomouc Czechia

Department of Clinical and Transplant Immunology Institute for Clinical and Experimental Medicine Prague Czechia

Department of Immunology 2nd Faculty of Medicine Charles University and Motol University Hospital Prague Czechia

Department of Immunology and Allergology Faculty of Medicine and Faculty Hospital in Pilsen Charles University Prague Pilsen Czechia

Department of Immunology and Allergy Institute of Health in Ostrava Ostrava Czechia

Department of Paediatrics 1st Faculty of Medicine Charles University Prague Prague Czechia

Institute of Clinical Immunology and Allergy Faculty of Medicine in Hradec Kralove University Hospital Hradec Kralove Charles University Hradec Kralove Czechia

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