Primary biliary cholangitis (PBC) is an autoimmune disease prevalent in middle-aged women and characterized by chronic cholestasis. PBC is diagnosed when at least two of the following three criteria are met: elevated alkaline phosphatase, presence of PBC-specific autoantibodies such as the anti-mitochondrial antibody or PBC-specific anti-nuclear antibodies, and non-suppurative inflammation of the interlobular bile duct after excluding other causes including drugs and biliary obstruction. The first-line treatment for PBC is ursodeoxycholic acid (UDCA, 13-15 mg/kg/day). The response to UDCA is predictive of long-term prognosis and should be evaluated 6-12 months after the UDCA treatment. The second-line treatments for PBC recommended due to an inadequate response to UDCA include obeticholic acid and fibrates. Symptoms and complications, including pruritus, sicca syndrome, and osteoporosis, should be evaluated and appropriately managed.
Keywords: Anti-mitochondrial antibody; Fibric acids; Liver cirrhosis, biliary; Obeticholic acid; Ursodeoxycholic acid.