Primary Biliary Cirrhosis

This chapter discusses clinical, pathologic, and epidemiologic features of Primary biliary cirrhosis (PBC). The diagnosis of PBC is based on three objective criteria. This triad consists of detectable serum antimitochondrial antibodies (AMA), increased enzymes indicating cholestasis for longer than 6 months, and a compatible or diagnostic liver histology. The chapter also describes the histology, autoimmune features, and epidemiology of PBC. It is noted that though genetics should be regarded as the major determinant in susceptibility to PBC, several other factors have been proposed, including a history of previous infections, comorbidity with other autoimmune diseases, and lifestyle factors, such as smoking and a high-fat diet. Furthermore, it is crucial to determine the pathogenic role of AMA in the bile duct damage of PBC. Once again, the development of an animal model appears to be the only way to provide a clear demonstration of such pathogenic mechanism. Finally, from a clinical standpoint, new clinical trials are needed to identify novel therapies in the long-term treatment of PBC.