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PBC and PSC

PSC: Pathogenesis

theory: unknown trigger (toxin, infection, ischemia) in a genetically susceptible host

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familial clustering reported

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class II and III HLA associations

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common autoantibodies (ANCA in 65-85%)

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humoral and cellular immune dysfunction

intra- and extra-hepatic bile duct inflammation and fibrosis

stricture formation and obliteration

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intra- and extra-hepatic ducts usually involved

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5% predominantly extra-hepatic

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25% predominantly intra-hepatic

leads to cholestasis and cirrhosis

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