Abstract
Primary biliary cirrhosis (PBC) is an autoimmune cholestatic liver disease. Susceptibility
to PBC probably arises from a combination of genetic and environmental factors. The
prevalence of PBC varies both on an international and a regional level. This can be
explained, in part, by differences in clinical practice and case-finding activity.
It is likely, however, that substantive geographical differences exist both in terms
of genetic susceptibility and environmental factors that potentially trigger the disease
in genetically susceptible individuals. The study of the epidemiology of PBC has strongly
supported the concept of an environmental triggering factor, but as yet no specific
agent has been identified. Ongoing work to discover the environmental agent, as well
as the mechanism that causes the disease will answer key questions as to the epidemiology
of this complex autoimmune disease as well as providing useful information for other
autoimmune conditions.
Keywords
primary biliary cirrhosis - epidemiology
