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Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned


PURPOSE OF REVIEW: The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has caused extreme concern for patients with inborn errors of immunity (IEIs). In the first 6 months of the pandemic, the case fatality rate among patients with IEIs resembled that of the general population (9%). This review aims at summarizing what we have learned about the course and outcome of coronavirus disease 2019 (COVID-19) in patients with different IEIs and what this can potentially teach us about the immune mechanisms that could confer protection or predisposition to severe disease. RECENT FINDINGS: A total of 649 patients with IEI and COVID-19 have been reported in the last year and a half, spanning all groups of the International Union of Immunological Societies classification of IEIs. For most patients, the underlying IEI does not represent an independent risk factor for severe COVID-19. In fact, some IEI may even be protective against the severe disease due to impaired inflammation resulting in less immune-mediated collateral tissue damage. SUMMARY: We review the characteristics of SARS-CoV-2 infection in a large number of patients with IEI. Overall, we found that combined immunodeficiencies, immune dysregulation disorders, and innate immune defects impairing type I interferon responses are associated with severe disease course.

Type Journal
ISBN 1531-698X (Electronic) 1040-8703 (Linking)
Authors Bucciol, G.; Tangye, S. G.; Meyts, I.
Responsible Garvan Author Prof Stuart Tangye
Published Date 2021-12-31
Published Volume 33
Published Issue 6
Published Pages 648-656
Status Published in-print
DOI 10.1097/MOP.0000000000001062
URL link to publisher's version