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Risk Factors for Severe COVID-19 and Hospital Admission in Patients With Inborn Errors of Immunity - Results From a Multicenter Nationwide Study

Tomáš Milota, Marta Sobotková, Jitka Smetanová, Markéta Bloomfield, Jana Vydláková, Zita Chovancová, Jiří Litzman, Roman Hakl, J Novák, Ivana Malkusová, Jana Hanzlı́ková, Dalibor Jílek, Beata Hutyrová, Vitezslav Novak, Irena Krčmová, Anna Šedivá, Pavlína Králíčková

2022Frontiers in Immunology33 citationsDOIOpen Access PDF

Abstract

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.

Topics & Concepts

MedicinePediatricsPopulationInternal medicineImmunologyEnvironmental healthImmunodeficiency and Autoimmune DisordersBlood disorders and treatmentsCystic Fibrosis Research Advances