Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency
© 2022 Campbell et al..
Autosomal recessive IRF7 deficiency was previously reported in three patients with single critical influenza or COVID-19 pneumonia episodes. The patients' fibroblasts and plasmacytoid dendritic cells produced no detectable type I and III IFNs, except IFN-β. Having discovered four new patients, we describe the genetic, immunological, and clinical features of seven IRF7-deficient patients from six families and five ancestries. Five were homozygous and two were compound heterozygous for IRF7 variants. Patients typically had one episode of pulmonary viral disease. Age at onset was surprisingly broad, from 6 mo to 50 yr (mean age 29 yr). The respiratory viruses implicated included SARS-CoV-2, influenza virus, respiratory syncytial virus, and adenovirus. Serological analyses indicated previous infections with many common viruses. Cellular analyses revealed strong antiviral immunity and expanded populations of influenza- and SARS-CoV-2-specific memory CD4+ and CD8+ T cells. IRF7-deficient individuals are prone to viral infections of the respiratory tract but are otherwise healthy, potentially due to residual IFN-β and compensatory adaptive immunity.
| Errataetall: | |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:219 |
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| Enthalten in: |
The Journal of experimental medicine - 219(2022), 7 vom: 04. Juli |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Campbell, Tessa Mollie [VerfasserIn] |
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| Links: |
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| Themen: |
Journal Article |
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| Anmerkungen: |
Date Completed 09.06.2022 Date Revised 16.05.2023 published: Print-Electronic ErratumIn: J Exp Med. 2022 Dec 5;219(12):. - PMID 36342405 Citation Status MEDLINE |
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| doi: |
10.1084/jem.20220202 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM341926450 |
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| 100 | 1 | |a Campbell, Tessa Mollie |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency |
| 264 | 1 | |c 2022 | |
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| 500 | |a Date Completed 09.06.2022 | ||
| 500 | |a Date Revised 16.05.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a ErratumIn: J Exp Med. 2022 Dec 5;219(12):. - PMID 36342405 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022 Campbell et al. | ||
| 520 | |a Autosomal recessive IRF7 deficiency was previously reported in three patients with single critical influenza or COVID-19 pneumonia episodes. The patients' fibroblasts and plasmacytoid dendritic cells produced no detectable type I and III IFNs, except IFN-β. Having discovered four new patients, we describe the genetic, immunological, and clinical features of seven IRF7-deficient patients from six families and five ancestries. Five were homozygous and two were compound heterozygous for IRF7 variants. Patients typically had one episode of pulmonary viral disease. Age at onset was surprisingly broad, from 6 mo to 50 yr (mean age 29 yr). The respiratory viruses implicated included SARS-CoV-2, influenza virus, respiratory syncytial virus, and adenovirus. Serological analyses indicated previous infections with many common viruses. Cellular analyses revealed strong antiviral immunity and expanded populations of influenza- and SARS-CoV-2-specific memory CD4+ and CD8+ T cells. IRF7-deficient individuals are prone to viral infections of the respiratory tract but are otherwise healthy, potentially due to residual IFN-β and compensatory adaptive immunity | ||
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| 700 | 1 | |a Zhang, Qian |e verfasserin |4 aut | |
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