The term ‘cholangiopathy’ refers to a chronic disorder which affects the ‘cholangiocytes’ or bile duct epithelial cells. The small cholangiocytes lining the intrahepatic bile ducts and the large cholangiocytes lining the extrahepatic bile ducts have varying functions, depending on their location in the biliary tree. These include production and transport of bile, remodelling of bile duct epithelium and repair of liver cell damage [1]. An injury or insult to cholangiocytes may evoke a series of responses in cholangiocytes which can eventually lead to periportal fibrosis, ductopaenia and biliary cirrhosis [1, 2]. Table 10.1