Cirrhosis and autoimmune liver disease: Current understanding

Table 1 Epidemiological characteristics associated with the three autoimmune liver diseases

	PBC	PSC	AIH
Female/male ratio	10/1	1/2	4/1
Average age at presentation	50	41	Childhood/adolescence and approximately 40
Incidence	0.33-5.8/100000	0-1.3/100000	0.08-3/100000
Prevalence	1.91-40.2/100000	0-16.2/100000	11.6-35.9/100000
Risk within family	1st degree relative incidence 4%-6%	Unknown	Unknown
Concordance in monozygotic twins	60%	Only case reports	Only case reports
Note	AMA positivity	Frequent association with IBD Increased risk of hepatobiliary/colorectal malignancies	Positivity for ANA and or SMA (AIH type-1) or anti-LKM-1 (AIH type-2)

AIH: Autoimmune hepatitis; AMA: Anti-mitochondrial autoantibodies; ANA: Anti-nuclear autoantibody; IBD: Inflammatory bowel disease; LKM: Liver kidney microsomal; PBC: Primary biliary cirrhosis; PSC: Primary sclerosing cholangitis; SMA: Smooth muscle autoantibody.