Liver immunity, autoimmunity, and inborn errors of immunity

Table 1 Characteristics of immune system components

	Innate immunity	Adaptive immunity
Cells	Macrophages, dendritic cells, neutrophils, eosinophiles, basophiles, mast cells, NK cells, γδT cells	T lymphocytes, B lymphocytes, NK cells, γδT cells
Molecules	Complement, cytokines, glycoproteins, chemokines, TLR, NLR, IL-1 beta, IL-18	Immunoglobulins, cytokines, chemokines
Response time	Rapid (minute-hour)	Slow (hour-days)
Response type	Response is non-spesific	Pathogen and antigen specific response
Memory	No immunological memory	Immunological memory
Dysregulated disease	Autoinflamatory diseases (e.g., periodic fever syndromes, systemic juvenile idiopatic artritis, adult onset Still disease, gout aritis)	Autoimmune diseases (e.g., mixed connective tissue diseases, systemic lupus erythnatosus, systemic sclerosis, idiopatic inflamatory myopaties, primary sjögren syndrome)

TKR: Toll-like receptors; NLR: Nod-like receptor; IL: Interleukin; NK: Natural killer.

Table 2 Mechanisms of autoimmunity

Item	Description
Exogen	Molecular mimicry
	Superantigen stimulation
	Microbial and tissue damage related adjuvant effect
Endogen	Loss of central and peripheral tolerance
	Autoreactive B and T lymphocytes
	Apoptotic defects and defects in cleaning apoptotic substance
	Disturbances in cytokine balance
	Change in immunoregulation

Table 3 Categories of inborn errors of immunity

No.	Inborn errors of immunity phenotypical classification
1	Immunodeficiencies affecting cellular and humoral immunity
2	Combined immunodeficiences with associated or syndromic features
3	Predominantly antibody deficiencies
4	Disease of immune dysregulation
5	Congenital defects of phagocyte number and function
6	Defects in intrinsic or innate immunity
7	Autoinflammatory disorders
8	Complement deficiencies
9	Phenocopies of inborn errors of immunity
10	Bone marrow failure