LAUSR

Purpose of review To discuss the most recent data regarding treatment of patients with primary biliary cholangitis (PBC) with inadequate response to ursodeoxycholic acid (UDCA). Recent findings Patients with PBC at high-risk of progressive disease are younger, have advanced fibrosis and showed inadequate response to UDCA after 12 months of treatment. These patients need a second-line treatment in addition to UDCA. The goal of therapy should be the normalization of ALP and bilirubin below 0.6 the upper limit of normal. Obeticholic acid (OCA) has proven to be effective in improving surrogate markers of prognosis in PBC, also in real-life cohort. Pruritus is the most frequent adverse event during treatment with OCA. Bezafibrate is another option in patients with inadequate response to UDCA as it was proven to improve surrogate endpoints, pruritus and even, clinical outcome compared with UDCA monotherapy. Finally, budesonide may be considered in patients with marked portal inflammation. Triple therapy with UDCA, OCA and bezafibrate may be considered in patients showing inadequate response to dual therapy. Summary Patients with PBC need to be evaluated at baseline, and on-treatment, for the risk of progressive disease and eventually treated with second-line therapies in addition to UDCA.