Immune-mediated bile duct injury: The case of primary biliary cirrhosis

Table 1 Hints towards an innate immunity involvement in primary biliary cirrhosis

	Peculiarity
Monocytes	Increase in absolute number
	Increase in pro-inflammatory cytokines upon infectious challenge
Elevated IgM	B cell response to bacterial stimuli
NKT cells	Increased NKT cells in PBC peripheral blood and liver
	Increased NKT cytotoxic activity
Liver histology	Focal duct obliteration with granuloma formation

PBC: primary biliary cirrhosis models.

Table 2 Features of spontaneous and induced murine primary biliary cirrhosis models resembling data in the human condition

	Mouse model	Adaptive immunity	Innate immunity
Spontaneous	Ae2(a.b)-deficient	AMA	--
		Lymphocytic CD8+ infiltrates
		Decreased T regulatory cells
		PBC-like liver lesions
	dnTGFbRII	AMA	NKT cells worsen liver injury
		Deficient T reg function
	IL2Ra-/-	AMA	--
		Portal tract CD4+ and CD8+ cells
	NOD.c3c4	Lymphocytic infiltrate	--
		AMA, ANA
Induced	N. aromaticivorans on NOD 1101	AMA	NKT cells are required
		PBC-like liver lesions
		Disease transfer by T cells
	Xenobiotic on C57BL/6	Lymphocytic CD8+ infiltrate	--
		AMA
		PBC-like liver lesions

AMA: antimitochondrial autoantibodies; ANA: antinuclear antibodies; NOD: non obese diabetic; PBC: primary biliary cirrhosis models.