The incidence of autoimmune diseases appears to be increasing in virtually every population. This is well illustrated by primary biliary cirrhosis, a disease characterized by an immune response to small bile ducts and an incidence increasing with age. The etiology of primary biliary cirrhosis remains enigmatic, although there is clearly an interplay of genetics and environment with a possible role for immunosenescence. The increasing usage of serologic tests for the hallmark of primary biliary cirrhosis, the antimitochondrial antibody, has dramatically changed the spectrum of disease presentation and natural history. In the past, data were predominantly based on the greater prevalence of advanced cases, whereas now most patients are detected earlier through the use of multi-phasic chemistry panels. In this review, we will place the epidemiology of primary biliary cirrhosis in the context of its clinical features and treatment as well as emphasize the future directions to further understand the immunobiology of this disease.
Copyright 2008 S. Karger AG, Basel.