COVID-19
Immunogenicity of Pfizer-BioNTech COVID-19 vaccine in patients with inborn errors of immunity

https://doi.org/10.1016/j.jaci.2021.05.029Get rights and content

Background

In mid-December 2020, Israel started a nationwide mass vaccination campaign against coronavirus disease 2019 (COVID-19). In the first few weeks, medical personnel, elderly citizens, and patients with chronic diseases were prioritized. As such, patients with primary and secondary immunodeficiencies were encouraged to receive the vaccine. Although the efficacy of RNA-based COVID-19 vaccines has been demonstrated in the general population, little is known about their efficacy and safety in patients with inborn errors of immunity (IEI).

Objective

Our aim was to evaluate the humoral and cellular immune response to COVID-19 vaccine in a cohort of patients with IEI.

Methods

A total of 26 adult patients were enrolled, and plasma and peripheral blood mononuclear cells were collected from them 2 weeks following the second dose of Pfizer-BioNTech COVID-19 vaccine. Humoral response was evaluated by testing anti–SARS-CoV-2 spike (S) receptor-binding domain and antinucleocapsid antibody titers and evaluating neutralizing ability by inhibition of receptor-binding domain–angiotensin-converting enzyme 2 binding. Cellular immune response was evaluated by using ELISpot, estimating IL-2 and IFN-γ secretion in response to pooled SARS-CoV-2 S- or M-peptides.

Results

Our cohort included 18 patients with a predominantly antibody deficiency, 2 with combined immunodeficiency, 3 with immune dysregulation, and 3 with other genetically defined diagnoses. Twenty-two of them were receiving immunoglobulin replacement therapy. Of the 26 patients, 18 developed specific antibody response, and 19 showed S-peptide–specific T-cell response. None of the patients reported significant adverse events.

Conclusion

Vaccinating patients with IEI is safe, and most patients were able to develop vaccine-specific antibody response, S-protein–specific cellular response, or both.

Key words

Inborn errors of immunity
IEI
primary immunodeficiency disorders
PIDD
SARS-CoV-2
COVID-19
vaccine
Pfizer-BioNTech
CVID
XLA
NFKB1
STAT1-GOF
STAT3-LOF
HIES
inhibiting antibodies

Abbreviations used

ACE2
Angiotensin-converting enzyme 2
ALPS
Autoimmune lymphoproliferative syndrome
anti-N
Antinucleocapsid
anti-S
Antispike
AU
Arbitrary unit
AUC
Area under the curve
CID
Combined immunodeficiency
COVID-19
Coronavirus disease 2019
CVID
Common variable immunodeficiency
IEI
Inborn errors of immunity
IVIG
Intravenous immunoglobulin
NF-κB1
Nuclear factor-κB1
NFKB1-HI
Nuclear factor-κB1 haploinsufficiency
RBD
Receptor-binding domain
SARS-CoV-2
Severe acute respiratory syndrome coronavirus 2
STAT
Signal transducer and activator of translation
STAT1-GOF
STAT1 gain-of-function
XLA
X-linked agammaglobulinemia

Cited by (0)

Supported by the Israel Science Foundation (grants 41222/18 and 3711/20 [to N.T.F.]), the Dahlia Greidinger Cancer Fund (to N.T.F.), a Marguerite Stolz Fellowship (N.T.F.), The Campbell Foundation for AIDS Research (to N.T.F.), and the Alrov Foundation (to D.H.).

Disclosure of potential conflict of interest: The authors declare that they have no relevant conflicts of interest.

These authors contributed equally to this work.

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