Cholestatic liver disease

Cholestatic liver disease remains a rare but increasingly recognized cause of illness affecting adults worldwide. Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two major cholestatic liver diseases. While there is much current evidence supporting an underlying immunologic process for both PBC and PSC, no unifying hypothesis exists to allow for the development of target-specific therapies. Because of advances in technology and the refinement of diagnostic modalities, an increasing number of individuals are being identified at earlier stages of disease who may benefit from existing treatment options. Despite improvements in the management of complications from end-stage liver disease and excellent long-term results from liver transplantation, a continued understanding of the clinico-pathologic processes responsible for cholestatic liver disease remains important.