What exactly is primary biliary cirrhosis?

Liver disease: Primary biliary cirrhosis is an autoimmune liver disease seen most commonly in women in the 4th to 5th decade of life. It generally presents with unexplained itching and occasionally jaundice. It is frequently found on routing screening liver tests which may show an elevated alkaline phosphotase. Though not curable it is treatable and treatment slows the progression to cirrhosis.

An inherited disease: Primary biliary cirrhosis is a condition of unknown cause with genetic predisposition, probably of an autoimmune nature, where the cells that line the biliary tract proliferate and eventually die and scarring occurs, which is what cirrhosis is about - scarring in the liver that interferes with its function. Definitive treatment is transplantation, but there are medications which can improve it.