Liver immunity, autoimmunity, and inborn errors of immunity

Area of Science:

• Immunology
• Hepatology

Background:

• The liver is a primary immune organ housing abundant phagocytic cells.
• It distinguishes self-antigens from non-self-antigens, like pathogens and food antigens.
• Liver immunity maintains a balance between immune screening and tolerance.

Purpose of the Study:

• To review the unique immunological features of the liver.
• To discuss dysregulated immune responses in autoimmune liver diseases (AILDs).
• To highlight the association between AILDs and inborn errors of immunity (IEIs).

Main Methods:

• Literature review focusing on liver immunity and autoimmune liver diseases.
• Analysis of the liver's anatomical and cellular structures influencing immune responses.
• Examination of the relationship between IEIs and AILDs.

Main Results:

• The liver's structure, including fenestrated endothelial cells and lack of basement membrane, facilitates immune cell-antigen interaction.
• Dynamic immune cell interactions are vital for maintaining liver immune homeostasis.
• Loss of immune tolerance can trigger autoimmune responses and liver diseases.

Conclusions:

• Liver immunity possesses unique properties, including antigen-specific tolerance induction.
• Dysregulated immune responses, particularly in the context of IEIs, are strongly linked to AILDs.
• Understanding liver immune dynamics is crucial for managing autoimmune and autoinflammatory liver conditions.