Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic
liver disease, which is invariably fatal. Circumstantial and indirect
evidence suggests that autoimmune mechanisms have a role in the pathog
enesis of PBC. Antimitochondrial antibodies (AMA) are highly sensitive
and specific markers that can predict the development of the disease
in a healthy individual. Long-term administration of ursodeoxycholic a
cid (UDCA), a naturally occurring bile acid, safely slows the progress
ion of PBC, delays the need for Liver transplantation, and postpones d
eath. An effort should be made to identify the patients with PBC in th
e asymptomatic stage by the presence of AMA and to conduct a clinical
trial in order to assess the benefit of long-term administration of UD
CA on the prevention of the overt disease in these individuals.