[Inflammatory and neoplastic cholangiopathies.]

The biliary tree consists of mature epithelial cells called cholangiocytes and is subdivided in intra and extrahepatic bile ducts. They facilitate the secretion and modification of the bile constituents and act as transport ducts of bile to the intestine. The alteration of the normal function of cholangiocyte, can lead to the development of multiple biliary diseases, known as cholangiopathies, generally chronic, with a progressive course and which often are lacking of an effective treatment, determining a poor prognosis, even lethal, for the patient. These cholangiopathies have peculiar characteristics both for onset and clinical course. The pathogenetic processes affecting cholangiocytes are not yet fully known. Depending on their nature, these diseases are further subdivided into genetic, idiopathic, which include primary biliary cholangitis, primary sclerosing cholangitis and associated IgG4 cholangitis and malignant such as cholangiocarcinoma or mixed hepato-cholangiocarcinoma. This review is focused on the new insights on the pathophysiological and molecular mechanisms involved in the liver damage cascade which provide the basis of novel therapeutic approaches for these cholangiopathies.