COVID-19 in a patient with Good's syndrome and in 13 patients with common variable immunodeficiency

Lindahl, Hannes; Smith, C. I. Edvard; Bergman, Peter

doi:10.1016/j.clicom.2021.08.003

Cited by 2 publications

(2 citation statements)

References 17 publications

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“…Patients with thymoma, a thymic epithelial tumor, 156 frequently develop T‐cell or Ab‐mediated autoimmune disease (including late‐onset myasthenia gravis, 157 and tissue‐specific auto‐Abs of the APS‐1 spectrum 158–160 ). They harbor auto‐Abs against IL‐17A/F, which are associated with the occurrence of the CMC, 38,158,159,161 and about ∼70% of patients have auto‐Abs against type I IFNs, 87,158,162 leading to a particularly high fatality rate for COVID‐19, especially in patients with Good syndrome (the association of thymoma and immunodeficiency) 163–173 . Interestingly, neoplastic mTECs do not express AIRE, 174,175 suggesting that the breakdown of T‐cell tolerance is a consequence of impaired central tolerance linked to impaired AIRE expression in the neoplastic tissue.…”

Section: Genetic Causes Of Auto‐abs Against Type I Ifnsmentioning

confidence: 99%

“…They harbor auto-Abs against IL-17A/F, which are associated with the occurrence of the CMC, 38,158,159,161 and about ∼70% of patients have auto-Abs against type I IFNs, 87,158,162 leading to a particularly high fatality rate for COVID-19, especially in patients with Good syndrome (the association of thymoma and immunodeficiency). [163][164][165][166][167][168][169][170][171][172][173] Interestingly, neoplastic mTECs do not express AIRE, 174,175 suggesting that the breakdown of T-cell tolerance is a consequence of impaired central tolerance linked to impaired AIRE expression in the neoplastic tissue.…”

Section: G Ene Ti CC Aus E Sofauto -Abs Ag Ain S T T Ype I Ifn Smentioning

confidence: 99%

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Human autoantibodies neutralizing type I IFNs: From 1981 to 2023

Bastard,

Gervais,

Le Voyer

et al. 2024

Immunological Reviews

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SummaryHuman autoantibodies (auto‐Abs) neutralizing type I IFNs were first discovered in a woman with disseminated shingles and were described by Ion Gresser from 1981 to 1984. They have since been found in patients with diverse conditions and are even used as a diagnostic criterion in patients with autoimmune polyendocrinopathy syndrome type 1 (APS‐1). However, their apparent lack of association with viral diseases, including shingles, led to wide acceptance of the conclusion that they had no pathological consequences. This perception began to change in 2020, when they were found to underlie about 15% of cases of critical COVID‐19 pneumonia. They have since been shown to underlie other severe viral diseases, including 5%, 20%, and 40% of cases of critical influenza pneumonia, critical MERS pneumonia, and West Nile virus encephalitis, respectively. They also seem to be associated with shingles in various settings. These auto‐Abs are present in all age groups of the general population, but their frequency increases with age to reach at least 5% in the elderly. We estimate that at least 100 million people worldwide carry auto‐Abs neutralizing type I IFNs. Here, we briefly review the history of the study of these auto‐Abs, focusing particularly on their known causes and consequences.

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Section: Genetic Causes Of Auto‐abs Against Type I Ifnsmentioning

confidence: 99%

Section: G Ene Ti CC Aus E Sofauto -Abs Ag Ain S T T Ype I Ifn Smentioning

confidence: 99%

Human autoantibodies neutralizing type I IFNs: From 1981 to 2023

Bastard,

Gervais,

Le Voyer

et al. 2024

Immunological Reviews

View full text Add to dashboard Cite

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Elevated CD21low B Cell Frequency Is a Marker of Poor Immunity to Pfizer-BioNTech BNT162b2 mRNA Vaccine Against SARS-CoV-2 in Patients with Common Variable Immunodeficiency

et al. 2022

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Purpose Limited data is available on the effect of COVID-19 vaccination in immunocompromised individuals. Here, we provide the results from vaccinating a single-center cohort of patients with common variable immunodeficiency (CVID). Methods In a prospective, open-label clinical trial, 50 patients with CVID and 90 age-matched healthy controls (HC) were analyzed for SARS-CoV-2 spike antibody (Ab) production after one or two doses of the Pfizer-BioNTech BNT162b2 mRNA vaccine. Additionally, in selected patients, SARS-CoV-2 spike-specific T-cells were assessed. Results A potent vaccine-induced anti-spike–specific IgG Ab response was observed in all the HC. In contrast, only 68.3% of the CVID patients seroconverted, with median titers of specific Ab being 83-fold lower than in HC. In fact, only 4/46 patients (8.6%) of patients who were seronegative at baseline reached the threshold for an optimal response (250 U/mL). Using the EUROclass definition, patients with either a reduced proportion, but not absolute counts, of switched memory B-cells or having an increased frequency of CD21low B-cells generally generated poor vaccine responses. Overall, CVID-patients had reduced spike-specific IFN-γ positive CD4+ T cell responses 2 weeks after the second dose, compared to HC. The total CD4 and CD4 central memory cell counts correlated with humoral immunity to the vaccine. Conclusions CVID patients with low frequency of switched memory B-cells or an increased frequency of CD21low B-cells according to the EUROclass definition demonstrated poor responses to Pfizer-BioNTech BNT162b2 mRNA vaccination. Cellular immune responses were significantly affected, affirming that the defect in CVID is not limited to humoral immunity.

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COVID-19 in a patient with Good's syndrome and in 13 patients with common variable immunodeficiency

Cited by 2 publications

References 17 publications

Human autoantibodies neutralizing type I IFNs: From 1981 to 2023

Human autoantibodies neutralizing type I IFNs: From 1981 to 2023

Elevated CD21low B Cell Frequency Is a Marker of Poor Immunity to Pfizer-BioNTech BNT162b2 mRNA Vaccine Against SARS-CoV-2 in Patients with Common Variable Immunodeficiency

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