Disease-modifying therapies and symptomatic management for primary biliary cholangitis.

Abstract

Primary biliary cholangitis is a chronic condition characterised by autoimmune destruction of intralobular bile ducts. Publications have shown widespread gaps in the care of patients with primary biliary cholangitis. This article reviews the literature regarding currently licensed first- and second-line therapies and evaluates therapeutic options for symptomatic management of primary biliary cholangitis. Ursodeoxycholic acid is recommended for all patients with primary biliary cholangitis, with obeticholic acid available as second-line therapy, both having demonstrated safety and efficacy. Potential disease-modifying therapies, such as fibrates and budesonide, require further investigation before licensing. Cholestyramine is first-line therapy for pruritus, albeit with limited evidence and common side-effects. There is no licensed therapy for primary biliary cholangitis-related fatigue; treating underlying causes where applicable is recommended. Disease-modifying and symptomatic therapies must be considered in tandem when managing patients with primary biliary cholangitis. Emerging therapies show initial promise but further randomised trials with long-term follow up are required to evaluate their efficacy as single or combination therapies.