Primary biliary cholangitis: primary autoimmune disease or primary secretory defect.

Expert Rev Gastroenterol Hepatol

Department of Pharmaceutical and Pharmacological Sciences, University of Padova, Padova, Italy.

Published: November 2023

Introduction: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by the immune-mediated destruction of small and medium intrahepatic bile ducts, involving predominantly females. PBC has long been described as an autoimmune liver disease, also because it is very often associated with many autoimmune conditions. More recently, another pathogenic mechanism exploring the damage of cholangiocytes has been hypothesized, i.e. a defect in the biliary umbrella which is physiologically responsible for the exchange of the ions Cl and HCO and maintains the integrity of glycocalyx. To provide a state-of-the-art analysis of this topic, a systematic review of literature in PubMed, Scopus, and Science Direct was conducted (inclusive dates: 1986-2023).

Area Covered: Although the etiology remains unknown, pathogenesis consists of a complex immune-mediated process resulting from a genetic susceptibility. PBC can be triggered by an immune-mediated response to an autoantigen, which leads to a progressive destruction of bile ducts and eventually to a progressive fibrosis with cirrhosis. The defect in the 'bicarbonate umbrella' acts as a protection against the toxic hydrophobic bile acids, leading to a toxic composition of bile.

Expert Opinion: This review offers a summary of the current knowledge about the pathogenesis of PBC, indicating that this is probably based on the mutual relationship between the immune insult and the unbalanced secretory mechanisms.

Download full-text PDF

Source
http://dx.doi.org/10.1080/17474124.2023.2242771DOI Listing

Publication Analysis

Top Keywords

primary biliary
8
biliary cholangitis
8
liver disease
8
bile ducts
8
primary
4
cholangitis primary
4
primary autoimmune
4
autoimmune disease
4
disease primary
4
primary secretory
4

Similar Publications

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!