Role of autoimmunity in primary biliary cirrhosis

Table 1 The characteristics of antinuclear antibodies in primary biliary cirrhosis patients

Staining pattern	Autoantigen	Prevalence (%)	Clinical significance
Nuclear dot	SP100[20,28]	25	Highly specific for PBC; Urinary tract infections
	PML[21,28]	19	Highly specific for PBC; Coexistence with anti-sp100
	SP140[22]	15	Coexistence with anti-sp100 and anti-PML
Nuclear periphery	gp210[23]	25	Association with disease severity and poor prognosis
(Nuclear pore complex)	p62[24]	30-55	Association with disease severity and poor prognosis
Nuclear periphery	Lamin[25]	6-8	Not highly specific for PBC
(Nuclear envelope)	LBR[24]	2-6	Highly specific for PBC
Anticentromere	Centromere[26]	30	Association with portal hypertension
?	AchR M3[27]	83	Unknown

PBC: Primary biliary cirrhosis; PML: Promyelocytic leukemia; LBR: Lamin B receptor.

Table 2 Differences in B cell depletion therapies in primary biliary cirrhosis murine models

Results	Model	Therapy (time)
Amelioration	NOD.c3c4 mice	Igμ knockout
	dnTGF-βRII mice	Anti-CD20 (4-6 wk)
Exacerbation	2OA-BSA-induced mice	Anti-CD20/Anti-CD79 (6 wk)
	dnTGF-βRII mice	Igμ knockout
No effect	dnTGF-βRII mice	Anti-CD20 (20-22 wk)

Table 3 The characteristics of primary biliary cirrhosis murine models and primary biliary cirrhosis patients

	NOD.c3c4[50]	dnTGF-βR II[59]	IL-2Rα(-/-)[58,85]	Ae2a,b(-/-)[86]	2OA-BSA induced[87]	Human
Disease onset	8-20 wk	4 wk	4 wk	6 mo?	4-12 wk	40-60 yr
AMA	56%	100%	100%	40-80%	100%	95%
ANA	80%-90%	(-)	80%	(-)	?	30%
Cytokines	Th1 ↑	Th1 ↑	Th1, Th17 ↑	Th1 ↑	Th1 ↑	Th1, Th17 ↑
Treg cells	↓	↓	↓	↓	↓	↓
Ig	IgG, IgM ↑	IgA ↑	IgG, IgA ↑	IgG, IgM ↑	?	IgM ↑
Liver inflammation
CD8+/CD4+T	↑	↑	↑	↑	↑	↑
Treg	?	↓	↓	↓	?	↑
Fibrosis	?	Slight	?	Slight	Slight	Evident in late stage
Complication	Bile cyst and	Colitis	Colitis	?	?	Thyroid disorder
	Extrahepatic damage		Anemia			Sjogren syndrome

ANA: Antinuclear antibodies; AMA: Antimitochondrial antibodies.