Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. Outcomes largely depend on early recognition of the disease and prompt institution of treatment. First-line treatment consists of ursodeoxycholic acid, but not all patients sufficiently respond to treatment. It is thus important to monitor patients for response to therapy to know when to initiate second-line treatment. The recent approval of PPAR agonists has greatly improved second-line treatment options, as these therapies not only elicited significantly better biochemical responses and normalized bilirubin levels but also improved extrahepatic symptoms including pruritus and fatigue, leading to better quality of life.