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. 2025 Feb;155(2):387-397.e6.
doi: 10.1016/j.jaci.2024.10.013. Epub 2024 Oct 22.

Inborn errors of immunity are associated with increased COVID-19-related hospitalization and intensive care compared to the general population

Hannes Lindahl  1 , Fredrik Kahn  2 , Åsa Nilsdotter-Augustinsson  3 , Mats Fredrikson  4 , Pontus Hedberg  5 , Isabela Killander Möller  5 , Lotta Hansson  6 , Lisa Blixt  6 , Sandra Eketorp Sylvan  7 , Anders Österborg  6 , Soo Aleman  8 , Christina Carlander  9 , Sofia Nyström  10 , Peter Bergman  11
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Free article

Inborn errors of immunity are associated with increased COVID-19-related hospitalization and intensive care compared to the general population

Hannes Lindahl et al. J Allergy Clin Immunol. 2025 Feb.
Free article

Abstract

Background: It is thought that patients with inborn errors of immunity (IEI) are more susceptible to severe coronavirus disease 2019 (COVID-19) than the general population, but a quantification of this potential risk is largely missing.

Objective: We assessed the impact of COVID-19 on patients with IEI.

Methods: A nationwide cohort study was performed to estimate the relative risk (RR) for hospitalization, intensive care, and death within 30 days after a positive severe acute respiratory syndrome coronavirus 2 test result in an IEI population (n = 2392) compared to the general population (n = 8,270,705) using data from Swedish national registries. Three time periods were studied: the prevaccination period, and the Alpha/Delta and Omicron periods. Adjustment was made for demographics, income, comorbidities, and vaccination status.

Results: During the prevaccination period, 25.2% of the IEI population was hospitalized, compared to 17.5% and 5.2% during the Alpha/Delta and Omicron periods, respectively. For the 3 time periods, the adjusted RR [95% confidence interval] for hospitalization in the IEI population compared to the general population was 3.1 [2.1-4.2], 3.5 [2.4-4.8], and 4.3 [2.5-6.7], respectively. The respective values for intensive care after COVID-19 were 5.6 [2.6-10.8], 4.7 [1.7-10.1], and 4.7 [1.7-10.1] for the 3 periods. Five patients (0.6%) in the IEI population died within 30 days of a positive PCR test result compared to 18,773 (0.2%) in the general population during the 3 study periods.

Conclusion: Patients with IEI had a 3 to 4 times higher risk for hospitalization and a 5 times higher risk for intensive care during COVID-19 compared to the general population.

Keywords: COVID-19; Inborn errors of immunity; cohort study; hospitalization; intensive care; population controls.

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Conflict of interest statement

Disclosure statement Funded by the Swedish Research Council (2021-04779). Disclosure of potential conflict of interest: S. Aleman reports a relationship with Gilead Sciences Inc that includes funding grants and speaking and lecture fees; a relationship with AbbVie Inc that includes funding grants and speaking and lecture fees; and a relationship with MSD that includes speaking and lecture fees; a relationship with Biogen that includes speaking and lecture fees; and has received a funding grant from Gilead with payment to Karolinska Institutet. C. Carlander reports a relationship with Gilead Sciences, ViiV, and MSD that includes consulting or advisory, funding grants, and speaking and lecture fees. L. Hansson reports a relationship with IQVIA that includes funding grants. P. Bergman reports a relationship with Takeda and CSL Behring that includes speaking and lecture fees. Anders Österborg has received independent grant support from Beigene, Merck/MSD, AstraZeneca, and Gilead. The rest of the authors declare that they have no relevant conflicts of interest.