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Review
. 2022 Nov;129(5):562-571.e1.
doi: 10.1016/j.anai.2022.06.009. Epub 2022 Jun 16.

Immunizing the imperfect immune system: Coronavirus disease 2019 vaccination in patients with inborn errors of immunity

Jessica R Durkee-Shock  1 , Michael D Keller  2
Affiliations
Review

Immunizing the imperfect immune system: Coronavirus disease 2019 vaccination in patients with inborn errors of immunity

Jessica R Durkee-Shock et al. Ann Allergy Asthma Immunol. 2022 Nov.

Abstract

Objective: To update clinicians on current evidence regarding the immunogenicity and safety of coronavirus disease 2019 (COVID-19) vaccines in patients with inborn errors of immunity (IEI).

Data sources: Peer-reviewed, published studies in PubMed, clinical trials listed on ClinicalTrials.gov, and professional organization and governmental guidelines.

Study selections: Literature searches on PubMed and ClinicalTrials.gov were performed using a combination of the following keywords: primary immunodeficiency, COVID-19, SARS-CoV-2, and vaccination.

Results: A total of 26 studies met the criteria and were included in this review. Overall, antibody responses to COVID-19 vaccination were found in 72% of study subjects, with stronger responses observed after messenger RNA vaccination. Neutralizing antibodies were detected in patients with IEI, though consistently at lower levels than healthy controls. Risk factors for poor antibody responses included diagnosis of common variable immunodeficiency, presence of autoimmune comorbidities, and use of rituximab. T cell responses were detectable in most patients with IEI, with poorer responses often found in patients with common variable immunodeficiency. Safety of COVID-19 vaccines in patients with IEI was acceptable with high rates of reactogenicity but very few serious adverse events, including in patients with immune dysregulation.

Conclusion: COVID-19 vaccines are safe in patients with IEI and seem to be immunogenic in most individuals, with stronger responses found after messenger RNA vaccinations.

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Figures

Figure 1
Figure 1
Overview of adaptive immune response to COVID-19 vaccination and potential disruption points for representative IEI. COVID-19, coronavirus disease 2019; CSR, class switch recombination; CVID, common variable immunodeficiency; IEI, inborn errors of immunity; IFN, interferon; Ig, immunoglobulin; IL, interleukin; SCID, severe combined immunodeficiency; SH, somatic hypermutation; TFH, T follicular helper; TNF, tumor necrosis factor.
Figure 2
Figure 2
Proposed workflow for active and passive COVID-19 immunization in patients with IEI. COVID-19, coronavirus disease 2019; IEI, inborn errors of immunity; mRNA, messenger RNA; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2.
Figure 3
Figure 3
COVID-19 vaccination in IEI: key points. CID, combined immunodeficiency; COVID-19, coronavirus disease 2019; CVID, common variable immunodeficiency; IEI, inborn errors of immunity; Ig, immunoglobulin; mRNA, messenger RNA; PAD, primary antibody deficiency.
See this image and copyright information in PMC

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