Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease with presumed autoimmune etiology. Current treatment options include ursodeoxycholic acid, obeticholic acid, and fibrate, which target mainly cholestasis. There is no effective therapy against autoimmune or hepatic fibrosis components. We can still achieve adequate biochemical response with monotherapy or a combination of medications in non-cirrhotic and compensated cirrhotic PBC patients. Several criteria are available for risk stratification and assess treatment response. Liver stiffness measurement by transient elastography is also a useful tool for evaluating disease progression. Lack of treatment or inadequate response are predictors of poor outcome. There is a strong need for additional therapies for PBC.