Ursodeoxycholic acid is the first line treatment for all patients with PBC. Response to treatment is assessed after 12 months of treatment using e.g. the Paris 1 criteria (ALP <3 times upper normal limit, AST <2 times upper normal limit, and bilirubin ≤1 mg/dL after one year of treatment). Up to 40% of patients respond inadequately to UDCA and need add-on therapies. (e.g. fibrates, budenosid or obeticholic acid).