Patients with autoimmune polyendocrine syndrome type 1 have an increased susceptibility to severe herpesvirus infections

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...

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Bibliographic Details
Published in:Clinical immunology (Orlando, Fla.) Fla.), 2021-10, Vol.231, p.108851-108851, Article 108851
Main Authors: Hetemäki, Iivo, Laakso, Saila, Välimaa, Hannamari, Kleino, Iivari, Kekäläinen, Eliisa, Mäkitie, Outi, Arstila, T. Petteri
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
AIRE
Autoantibody
Child
Cohort Studies
Female
Full Length
Herpes simplex
Herpes zoster
Herpesvirus 3, Human
Humans
Immunity, Cellular
Immunodeficiency
Interferon-alpha - immunology
Male
Middle Aged
Polyendocrinopathies, Autoimmune - complications
Polyendocrinopathies, Autoimmune - immunology
Risk Factors
Type I interferon
Varicella zoster
Varicella Zoster Virus Infection - complications
Varicella Zoster Virus Infection - pathology
Young Adult
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Summary: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-IFNα4 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. •Patients with APS-1 have high incidence of VZV reactivation.•VZV reactivations are associated with high neutralizing anti-interferon antibody titers.•The patients have decreased humoral immune responses to varicella zoster virus.•The patients often present with severe clinical sequelae of herpesvirus infections.
ISSN:1521-6616
1521-7035
DOI:10.1016/j.clim.2021.108851