Primary and Acquired Immunodeficiencies Associated with Severe Varicella-Zoster Infections.
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ABSTRACT: Background
While most cases of varicella or zoster are self-limited, patients with certain immune deficiencies may develop severe or life-threatening disease.Methods
We studied a patient with varicella-zoster virus (VZV) CNS vasculopathy and as part of the evaluation, tested his plasma for antibodies to cytokines. We reviewed the literature for cases of varicella or zoster associated with primary and acquired immunodeficiencies.Results
We found that a patient with VZV CNS vasculopathy had antibody that neutralized interferon (IFN)-α, but not IFN-γ. The patient's plasma blocked phosphorylation in response to stimulation with IFN-α in healthy control peripheral blood mononuclear cells. In addition to acquired immunodeficiencies like HIV or autoantibodies to IFN, variants in specific genes have been associated with severe varicella and/or zoster. While these genes encode proteins with very different activities, many affect IFN signaling pathways, either those that sense double-stranded RNA or cytoplasmic DNA that trigger IFN production, or those involved in activation of IFN stimulated genes in response to binding of IFN with its receptor.Conclusions
Immune deficiencies highlight the critical role of IFN in control of VZV infections and suggest new approaches for treatment of VZV infection in patients with certain immune deficiencies.
SUBMITTER: Ansari R
PROVIDER: S-EPMC8563170 | biostudies-literature |
REPOSITORIES: biostudies-literature
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