Changing Nomenclature for PBC from “Primary Biliary Cirrhosis” to “Primary Biliary Cholangitis”

Primary biliary cirrhosis (PBC) Primary sclerosing cholangitis (PSC)

Primary sclerosing cholangitis and primary biliary cirrhosis.

Preview Although primary sclerosing cholangitis occurs most often in middle-aged or younger men and primary biliary cirrhosis in middle-aged or older women, the diseases have some traits in common-unfortunate ones. In both diseases, the cause is unknown, and liver transplantation is the only known effective therapy for advanced cases. Dr Fennerty discusses trials of medical treatments used in a...

Impact of Microbes on the Pathogenesis of Primary Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC)

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent the major clinical entities of chronic cholestatic liver diseases. Both disorders are characterized by portal inflammation and slowly progress to obliterative fibrosis and eventually liver cirrhosis. Although immune-pathogenic mechanisms have been implicated in the pathogenesis of PBC and PSC, neither disorder is...

Current management of primary biliary cirrhosis and primary sclerosing cholangitis.

Primary biliary cirrhosis (PBC) is a chronic, cholestatic autoimmune liver disease characterized by inflammation and progressive destruction of interlobular bile ducts, ultimately leading to biliary cirrhosis. Population based studies have estimated the incidence of PBC as 19.1–251/ 1 000 000 in the general population [1,2]. The etiology of PBC is attributed to autoimmunity mainly due to the as...

Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis.

Treatment of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) with ursodeoxycholic acid (UDCA) has been in common use since 1985. In PBC, treatment with UDCA improves laboratory data, liver histology, enables a longer transplantation-free interval and prolongs disease survival. Because UDCA is unable to cure the disease newer drugs or combination therapies are still need...