Published online Aug 27, 2020. doi: 10.4254/wjh.v12.i8.423
Peer-review started: May 6, 2020
First decision: May 24, 2020
Revised: June 7, 2020
Accepted: August 1, 2020
Article in press: August 1, 2020
Published online: August 27, 2020
Processing time: 110 Days and 10.3 Hours
Cholestatic liver diseases (CLD) begin to develop after an impairment of bile flow start to affect the biliary tree. Cholangiocytes actively participate in the liver response to injury and repair and the intensity of this reaction is a determinant factor for the development of CLD. Progressive cholangiopathies may ultimately lead to end-stage liver disease requiring at the end orthotopic liver transplantation. This narrative review will discuss cholangiocyte biology and pathogenesis mechanisms involved in four intrahepatic CLD: Primary biliary cholangitis, primary sclerosing cholangitis, cystic fibrosis involving the liver, and polycystic liver disease.
Core tip: Several factors can condition bile flow derangements including environmental triggering factors, bile transport obstruction and conditions that alter bile concentration. Sustained pro inflammatory signaling associated with genetic and/or epigenetic dysregulation can condition a chronic dysfunctional state that can lead to a fibrogenic state with loss of homeostasis and sometimes malignant transformation.