Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency.
J Exp Med
; 219(7)2022 07 04.
Article
in English
| MEDLINE | ID: covidwho-1878728
ABSTRACT
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-ß. 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-ß and compensatory adaptive immunity.
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Viruses
/
Virus Diseases
/
Influenza, Human
/
COVID-19
Type of study:
Prognostic study
Topics:
Variants
Limits:
Adult
/
Humans
Language:
English
Year:
2022
Document Type:
Article
Affiliation country:
Jem.20220202
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