Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency
- PMID: 35670811
- PMCID: PMC9178406
- DOI: 10.1084/jem.20220202
Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency
Erratum in
-
Correction: Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency.J Exp Med. 2022 Dec 5;219(12):e2022020210282022c. doi: 10.1084/jem.2022020210282022c. Epub 2022 Nov 7. J Exp Med. 2022. PMID: 36342405 Free PMC article. No abstract available.
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-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.
© 2022 Campbell et al.
Conflict of interest statement
Disclosures: J.-L. Casanova reported a patent to PCT/US2021/042741 pending. I. Meyts reported grants from CSL Behring—paid to KU Leuven outside the submitted work. No other disclosures were reported.
Figures








References
-
- Abolhassani, H., Landegren N., Bastard P., Materna M., Modaresi M., Du L., Aranda-Guillen M., Sardh F., Zuo F., Zhang P., et al. . 2022. Inherited IFNAR1 deficiency in a child with both critical COVID-19 pneumonia and multisystem inflammatory syndrome. J. Clin. Immunol. 42:471–483. 10.1007/s10875-022-01215-7 - DOI - PMC - PubMed
-
- Asano, T., Boisson B., Onodi F., Matuozzo D., Moncada-Velez M., Maglorius Renkilaraj M.R.L., Zhang P., Meertens L., Bolze A., Materna M., et al. . 2021. X-linked recessive TLR7 deficiency in ∼1% of men under 60 years old with life-threatening COVID-19. Sci. Immunology. 6:eabl4348. 10.1126/sciimmunol.abl4348 - DOI - PMC - PubMed
Publication types
MeSH terms
Grants and funding
LinkOut - more resources
Full Text Sources
Medical
Research Materials
Miscellaneous