Dendritic cells in inborn errors of immunity.

Area of Science:

• Immunology
• Genetics
• Cell Biology

Background:

• Dendritic cells (DCs) are central to initiating and maintaining immune responses, impacting homeostasis, inflammation, and autoimmunity.
• Inborn errors of immunity (IEI) encompass 465 genetic disorders, often involving molecules that regulate DC function.
• Comprehensive studies on DCs in IEI are limited, despite their critical roles.

Purpose of the Study:

• To review the biology of dendritic cells (DCs) in inborn errors of immunity (IEI).
• To analyze DC alterations in IEI based on the recent International Union of Immunological Societies (IUIS) classification.
• To correlate DC dysfunction with clinical and immunological manifestations in selected IEI.

Main Methods:

• Literature review of dendritic cell biology in inborn errors of immunity.
• Analysis based on the International Union of Immunological Societies (IUIS) classification of IEI.
• In-depth discussion of DC changes in IEI with available clinical and immunological data.

Main Results:

• Dendritic cell (DC) function is significantly altered in various inborn errors of immunity (IEI).
• Specific IEI categories, including severe immunodeficiencies and disorders of synapse formation, show notable DC abnormalities.
• DC dysregulation contributes to the clinical and immunological phenotypes observed in these IEI.

Conclusions:

• Dendritic cells play a critical role in the pathogenesis of numerous inborn errors of immunity.
• Understanding DC biology in IEI is essential for diagnosing and managing these complex disorders.
• Further research into DC function across all IEI categories is warranted.