Adult-Onset Immunodeficiency in Thailand and Taiwan
Patients in Thailand and Taiwan without a known immunodeficiency who had serious infections with nontuberculous mycobacteria and other opportunistic infections were identified. Immunologic evaluation showed a strong association with autoantibodies to interferon-γ. The control of infection with mycob...
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Veröffentlicht in: | The New England journal of medicine 2012-08, Vol.367 (8), p.725-734 |
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Sprache: | eng |
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Zusammenfassung: | Patients in Thailand and Taiwan without a known immunodeficiency who had serious infections with nontuberculous mycobacteria and other opportunistic infections were identified. Immunologic evaluation showed a strong association with autoantibodies to interferon-γ.
The control of infection with mycobacteria, dimorphic molds, and salmonella depends on the integrity of the interferon-γ, interleukin-12, and tumor necrosis factor α (TNF-α) pathways, as shown by mendelian defects and iatrogenic inhibition of these pathways.
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Patients with genetic defects tend to present early in life, and the defects often show familial clustering, whereas patients with therapeutic antibody–associated disease have clear risk factors and tend to present in adulthood.
Anticytokine autoantibodies are increasingly recognized as having a role in disease pathogenesis, including susceptibility to infection.
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Since 2004, disseminated nontuberculous mycobacterial and other opportunistic infections involving neutralizing anti–interferon-γ autoantibodies . . . |
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ISSN: | 0028-4793 1533-4406 |
DOI: | 10.1056/NEJMoa1111160 |