Abstract
We demonstrate that autosomal-dominant STAT3-deficient hyper-IgE syndrome confers late-onset susceptibility to molds, especially Aspergillus, despite preserved myeloid functions, in association with previous parenchymal lung damage (bronchiectasis/pneumatoceles). This suggests a critical role for non-hematopoietic processes in innate antifungal immunity in AD-HIES.
Publication types
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Letter
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Research Support, N.I.H., Intramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Age of Onset
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Aspergillus / immunology*
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Aspergillus / pathogenicity
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Bronchiectasis
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Cells, Cultured
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Disease Susceptibility
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Genetic Association Studies
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Humans
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Immunoglobulin E / genetics
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Immunoglobulin E / immunology
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Immunoglobulin E / metabolism*
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Invasive Pulmonary Aspergillosis / epidemiology
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Invasive Pulmonary Aspergillosis / genetics
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Invasive Pulmonary Aspergillosis / immunology*
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Invasive Pulmonary Aspergillosis / physiopathology
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Job Syndrome / epidemiology
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Job Syndrome / genetics
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Job Syndrome / immunology*
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Job Syndrome / physiopathology
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Mutation / genetics
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STAT3 Transcription Factor / genetics*
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STAT3 Transcription Factor / immunology
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Survival Analysis
Substances
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STAT3 Transcription Factor
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Immunoglobulin E