In the future, the agents which are currently being tested in cholestatic and fibrotic liver diseases in adults (132) can also be investigated in BA, such as the farnesoid X receptor (FXR) agonist, obeticholic acid, and the modified bile acid norursodeoxycholic acid, which are also currently used in primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) patients (133, 134). This evidence concerns the gene NR1H4 and primary biliary cholangitis.