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Clinical Reviews in Allergy & Immunology
Published in: Clinical Reviews in Allergy & Immunology 1/2022

21-08-2021 | Herpes Simplex Encephalitis

Defects of the Innate Immune System and Related Immune Deficiencies

Author: Nicole Akar-Ghibril

Published in: Clinical Reviews in Allergy & Immunology | Issue 1/2022

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Abstract

The innate immune system is the host’s first line of defense against pathogens. Toll-like receptors (TLRs) are pattern recognition receptors that mediate recognition of pathogen-associated molecular patterns. TLRs also activate signaling transduction pathways involved in host defense, inflammation, development, and the production of inflammatory cytokines. Innate immunodeficiencies associated with defective TLR signaling include mutations in NEMO, IKBA, MyD88, and IRAK4. Other innate immune defects have been associated with susceptibility to herpes simplex encephalitis, viral infections, and mycobacterial disease, as well as chronic mucocutaneous candidiasis and epidermodysplasia verruciformis. Phagocytes and natural killer cells are essential members of the innate immune system and defects in number and/or function of these cells can lead to recurrent infections. Complement is another important part of the innate immune system. Complement deficiencies can lead to increased susceptibility to infections, autoimmunity, or impaired immune complex clearance. The innate immune system must work to quickly recognize and eliminate pathogens as well as coordinate an immune response and engage the adaptive immune system. Defects of the innate immune system can lead to failure to quickly identify pathogens and activate the immune response, resulting in susceptibility to severe or recurrent infections.
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Metadata
Title
Defects of the Innate Immune System and Related Immune Deficiencies
Author
Nicole Akar-Ghibril
Publication date
21-08-2021
Publisher
Springer US
Published in
Clinical Reviews in Allergy & Immunology / Issue 1/2022
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-021-08885-y

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