Infections by varicella zoster virus in patients with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
Journal of Allergy and Clinical Immunology
; 151(2):AB211, 2023.
Article
in English
| EMBASE | ID: covidwho-2243855
ABSTRACT
Rationale APECED is a life-threatening monogenic disorder characterized by multiorgan autoimmunity. Most patients harbor autoantibodies (auto-abs) to type-1 interferons (IFNs), which are important mediators of viral defense. Auto-abs to type-1 IFNs are associated with severe COVID-19 and may play a role in other viral infections including by varicella zoster virus (VZV). A recent study of 44 European APECED patients reported increased susceptibility to VZV, and a correlation between VZV recurrence and auto-abs to IFN-α. The clinical, immunophenotypic, and auto-ab characteristics of APECED patients in the USA with VZV are described. Methods:
Data was obtained from 103 participants on a prospective, natural history study after informed consent. Auto-abs to IFN-α, IFN-β or IFN-ω were measured using a multiplex particle-based assay. Unpaired t tests or U Mann Whitney tests were used, with Holm correction for multiple comparisons, where appropriate.Results:
Twenty-six patients reported childhood chickenpox (mean onset 5.6 years) and 2 (7.7%) required hospitalization for severe disease. Nineteen patients (18%) had at least one episode of shingles (median onset 13 years;range, 4-55 years) and 4 had >1 episode. Fifteen of the 19 patients with shingles (79%) were not receiving immunosuppressive medications during their first infection. VZV IgG levels;total and percent CD3, CD8, CD4, CD19, NK cells;and auto-abs to IFN-α, IFN-β and IFN-ω did not significantly differ between patients with or without recurrent shingles.Conclusions:
A subset of APECED patients develop early-onset, recurrent VZV infections even in the absence of immunosuppression. The mechanisms underlying susceptibility to VZV in APECED require further study.
alpha interferon; autoantibody; beta interferon; CD19 antigen; CD3 antigen; CD4 antigen; CD8 antigen; endogenous compound; immunoglobulin G; omega interferon; adolescent; adult; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy; chickenpox; child; childhood; conference abstract; controlled study; female; gene expression; herpes zoster; history; hospitalization; human; immunoglobulin blood level; immunosuppressive treatment; informed consent; major clinical study; male; natural killer cell; nonhuman; prospective study; rank sum test; Varicella zoster virus
Full text:
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Collection:
Databases of international organizations
Database:
EMBASE
Language:
English
Journal:
Journal of Allergy and Clinical Immunology
Year:
2023
Document Type:
Article
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