Are All Primary Immunodeficiency Disorders Inborn Errors of Immunity?

Ameratunga, Rohan; Longhurst, Hilary; Lehnert, Klaus; Steele, Richard; Edwards, Emily S.J.; Woon, See‐Tarn

doi:10.3389/fimmu.2021.706796

Cited by 8 publications

(2 citation statements)

References 40 publications

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“…Patients with primary immunodeficiency disorders have provided valuable insights into the role of various components of the immune system in the protective response to SARS-CoV-2. 24 Current data indicate that patients with innate immune system or T-cell defects have worse outcomes, attesting to the importance of these components of the immune response. 25 In contrast, most patients with X-linked agammaglobulinemia (XLA), without comorbidities, appear to be protected from severe disease.…”

Section: Sars-cov-2 and Immunodeficiency Disordersmentioning

confidence: 99%

SARS-CoV-2 Omicron: Light at the End of the Long Pandemic Tunnel or Another False Dawn for Immunodeficient Patients?

Ameratunga

Leung

Woon

et al. 2022

The Journal of Allergy and Clinical Immunology: In Practice

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Section: Sars-cov-2 and Immunodeficiency Disordersmentioning

confidence: 99%

SARS-CoV-2 Omicron: Light at the End of the Long Pandemic Tunnel or Another False Dawn for Immunodeficient Patients?

Ameratunga

Leung

Woon

et al. 2022

The Journal of Allergy and Clinical Immunology: In Practice

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“…Patients with primary immunodeficiency disorders (PIDs) missing specific components of the immune system have been termed ‘experiments of nature’ 19. Most, but not all, have inborn errors of immunity (IEIs) caused by genetic defects 20. Analysis of infections in patients with PID has shown the pattern of disease susceptibility is associated with specific immune defects.…”

Section: Introductionmentioning

confidence: 99%

Critical role of diagnostic SARS-CoV-2 T cell assays for immunodeficient patients

et al. 2022

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After almost 3 years of intense study, the immunological basis of COVID-19 is better understood. Patients who suffer severe disease have a chaotic, destructive immune response. Many patients with severe COVID-19 produce high titres of non-neutralising antibodies, which are unable to sterilise the infection. In contrast, there is increasing evidence that a rapid, balanced cellular immune response is required to eliminate the virus and mitigate disease severity. In the longer term, memory T cell responses, following infection or vaccination, play a critical role in protection against SARS-CoV-2.Given the pivotal role of cellular immunity in the response to COVID-19, diagnostic T cell assays for SARS-CoV-2 may be of particular value for immunodeficient patients. A diagnostic SARS-CoV-2 T cell assay would be of utility for immunocompromised patients who are unable to produce antibodies or have passively acquired antibodies from subcutaneous or intravenous immunoglobulin (SCIG/IVIG) replacement. In many antibody-deficient patients, cellular responses are preserved. SARS-CoV-2 T cell assays may identify breakthrough infections if reverse transcriptase quantitative PCR (RT-qPCR) or rapid antigen tests (RATs) are not undertaken during the window of viral shedding. In addition to utility in patients with immunodeficiency, memory T cell responses could also identify chronically symptomatic patients with long COVID-19 who were infected early in the pandemic. These individuals may have been infected before the availability of reliable RT-qPCR and RAT tests and their antibodies may have waned. T cell responses to SARS-CoV-2 have greater durability than antibodies and can also distinguish patients with infection from vaccinated individuals.

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Severe Adverse Reaction to Measles Vaccine Due to Homozygous Mutation in the IFNAR2 Gene: A Case Report and Literature Review

Adi,

Obaid,

Hafez

et al. 2024

J Clin Immunol

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Are All Primary Immunodeficiency Disorders Inborn Errors of Immunity?

Cited by 8 publications

References 40 publications

SARS-CoV-2 Omicron: Light at the End of the Long Pandemic Tunnel or Another False Dawn for Immunodeficient Patients?

SARS-CoV-2 Omicron: Light at the End of the Long Pandemic Tunnel or Another False Dawn for Immunodeficient Patients?

Critical role of diagnostic SARS-CoV-2 T cell assays for immunodeficient patients

Severe Adverse Reaction to Measles Vaccine Due to Homozygous Mutation in the IFNAR2 Gene: A Case Report and Literature Review

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