Bile Acids and Biliary Fibrosis

Biliary fibrosis is the driving pathological process in cholangiopathies such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Cholangiopathies are also associated with cholestasis, which is the retention of biliary components, including bile acids, in the liver and blood. Cholestasis may worsen with biliary fibrosis. Furthermore, bile acid levels, composition and homeostasis are dysregulated in PBC and PSC. In fact, mounting data from animal models and human cholangiopathies suggest that bile acids play a crucial role in the pathogenesis and progression of biliary fibrosis. The identification of bile acid receptors has advanced our understanding of various signaling pathways involved in regulating cholangiocyte functions and the potential impact on biliary fibrosis. We will also briefly review recent findings linking these receptors with epigenetic regulatory mechanisms. Further detailed understanding of bile acid signaling in the pathogenesis of biliary fibrosis will uncover additional therapeutic avenues for cholangiopathies.

Automated summary

Biliary fibrosis is the key pathological process in cholangiopathies, and cholestasis worsens with biliary fibrosis. Dysregulation of bile acid levels and composition is observed in PBC and PSC. Recent studies have shown that bile acids play a crucial role in the progression of biliary fibrosis, and the identification of bile acid receptors has advanced our understanding of related signaling pathways. Further understanding of bile acid signaling may lead to new therapeutic approaches for cholangiopathies.