Primary biliary cirrhosis (PBC) is a chronic autoimmune disease characteris
ed by cholestatic liver function tests, antimitochondrial antibodies, and a
bnormal liver histology. Early descriptions of a rare rapidly progressive d
isease no longer reflect the more indolent progress often seen today. Many
patients have significant long term morbidity through symptoms such as fati
gue and itch with a minority progressing to liver failure and need for tran
splantation. The current data on the diagnosis, clinical progression, and t
reatment of PBC are reviewed.