Journal
CLINICAL IMMUNOLOGY
Volume 231, Issue -, Pages -Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2021.108851
Keywords
AIRE; Herpes simplex; Varicella zoster; Herpes zoster; Autoantibody; Immunodeficiency; Type I interferon
Categories
Funding
- Helsinki University research funds
- Helsinki University Doctoral Programme in Biomedicine
- Sigrid Juselius Foundation
- European Joint Program for Rare Diseases and Academy of Finland [334813, 334812]
- Novo Nordisk Foundation
- Foundation for Pediatric Research
- Swedish Childhood Cancer Foundation
- Academy of Finland (AKA) [334812, 334813, 334812, 334813] Funding Source: Academy of Finland (AKA)
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The study revealed that higher titers of neutralizing anti-IFN alpha 4 antibodies in APS-1 patients are associated with a higher and earlier incidence of VZV reactivation, leading to more severe clinical sequelae of herpetic infections. These results suggest that blocking the type I interferon pathway in APS-1 patients results in a clinically significant immune deficiency, emphasizing the importance of considering susceptibility to herpesviruses when treating APS-1 patients.
Almost all patients with autoimmune polyendocrine syndrome type 1 (APS-1) have neutralizing antibodies against type 1 interferons (IFN), important mediators of antiviral defense. Recently, neutralizing anti-IFN antibodies were shown to be a risk factor of severe COVID-19. Here we show in a cohort of 44 patients with APS-1 that higher titers of neutralizing anti-IFN alpha 4 antibodies are associated with a higher and earlier incidence of VZV reactivation (herpes zoster). The patients also present with uncommonly severe clinical sequelae of herpetic infections. APS-1 patients had decreased humoral immune responses to varicella zoster virus, but cellular responses were comparable to healthy controls. These results suggest that blocking the type I interferon pathway in patients with APS-1 patients leads to a clinically significant immune deficiency, and susceptibility to herpesviruses should be taken into account when treating patients with APS-1.
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