Inborn errors of immunity are associated with increased COVID-19–related hospitalization and intensive care compared to the general population - 15/11/24

Doi : 10.1016/j.jaci.2024.10.013

Hannes Lindahl, MD, PhD ^a,^b, Fredrik Kahn, MD, PhD ^c,^d, Åsa Nilsdotter-Augustinsson, MD, PhD ^e, Mats Fredrikson, PhD ^f, Pontus Hedberg, MD, PhD ^g, Isabela Killander Möller, MD ^g, Lotta Hansson, MD, PhD ^h,ⁱ, Lisa Blixt, MD ^h,ⁱ, Sandra Eketorp Sylvan, MD, PhD ⁱ, Anders Österborg, MD, PhD ^h,ⁱ, Soo Aleman, MD, PhD ^j,^k, Christina Carlander, MD, PhD ^j,^k,^l, Sofia Nyström, MD, PhD ^m,ⁿ, Peter Bergman, MD, PhD ^a,^k,^o,^⁎
^a Department of Clinical Immunology and Transfusion Medicine, Karolinska University Hospital, Stockholm, Sweden
^b Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden
^c Department of Clinical Sciences Lund, Section for Infection Medicine, Lund University, Lund, Sweden
^d Department of Infectious Diseases, Skåne University Hospital, Lund, Sweden
^e Division of Inflammation and Infection, Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
^f Department of Biomedical and Clinical Sciences, Faculty of Medicine, Linköping University, Linköping, Sweden
^g Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden
^h Department of Hematology, Karolinska University Hospital, Stockholm, Sweden
ⁱ Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
^j Division of Infectious Diseases and Dermatology, Department of Medicine Huddinge, Karolinska Institutet, Stockholm, Sweden
^k Department of Infectious Diseases, Karolinska University Hospital, Stockholm, Sweden
^l Department of Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden
^m Department of Clinical Immunology and Transfusion Medicine, Linköping University, Linköping, Sweden
ⁿ Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
^o Department of Laboratory Medicine, Karolinska Institutet, Stockholm, Sweden

^∗Corresponding author: Peter Bergman, MD, PhD, Department of Laboratory Medicine, Karolinska Institutet, 141 52 Huddinge, Sweden.Department of Laboratory MedicineKarolinska InstitutetHuddinge141 52Sweden

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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.

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Key words : Inborn errors of immunity, COVID-19, cohort study, population controls, hospitalization, intensive care

Abbreviations used : adjRR, ATC, BMI, CI, COVID-19, CVID, ICU, IEI, IgRT, LISA, NPR, NVR, PIDcare, RR, SARS-CoV-2, SCDR, SIR, SmiNet, SPDR, TPR