Yokoda RT, Rodriguez EA. Review: Pathogenesis of cholestatic liver diseases. World J Hepatol 2020; 12(8): 423-435 [PMID: 32952871 DOI: 10.4254/wjh.v12.i8.423]
Corresponding Author of This Article
Eduardo A Rodriguez, FACP, MD, Assistant Professor, Department of Gastroenterology, Hepatology and Nutrition, University of Utah, 30 N 1900 E, Room 4R118, Salt Lake City, UT 84132, United States. eduardo.rodriguez@hsc.utah.edu
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Hepatol. Aug 27, 2020; 12(8): 423-435 Published online Aug 27, 2020. doi: 10.4254/wjh.v12.i8.423
Review: Pathogenesis of cholestatic liver diseases
Raquel T Yokoda, Eduardo A Rodriguez
Raquel T Yokoda, Department of Anatomic and Clinical Pathology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, United States
Eduardo A Rodriguez, Department of Gastroenterology, Hepatology and Nutrition, University of Utah, Salt Lake City, UT 84132, United States
Author contributions: Yokoda RT and Rodriguez EA wrote the paper and approved the final version.
Conflict-of-interest statement: Authors declare no conflict of interests for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Eduardo A Rodriguez, FACP, MD, Assistant Professor, Department of Gastroenterology, Hepatology and Nutrition, University of Utah, 30 N 1900 E, Room 4R118, Salt Lake City, UT 84132, United States. eduardo.rodriguez@hsc.utah.edu
Received: May 6, 2020 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
Abstract
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.
