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PBC and PSC

PBC: Natural History and Prognosis

AMA positive with normal LFTs and asymptomatic

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29 patients followed (18 years average)

24 with PBC on biopsy (83%)

2 with normal biopsies

24 developed abnormal LFTs at average of 5.6 years

22 developed symptomatic disease

no progression to cirrhosis or death from liver disease

asymptomatic PBC (AMA and biopsy) with abnormal LFTs

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40% develop symptoms in 5-7 years

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once symptomatic, life expectancy falls (median survival 10 years but a large range)

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