Diagnosing and treating a patient with primary biliary cirrhosis

Primary biliary cirrhosis is a chronic cholestatic disease of unknown aetiology. It is believed to be of autoimmune origin, primarily affecting middle-aged women. The disease is typically characterized by progressive damage to intrahepatic bile ducts, leading to liver fibrosis and eventual cirrhosis. It accounts for 2% of all cirrhosis deaths worldwide. Disease symptoms are varied, with many patients being asymptomatic in the early disease stages, which is reflected by the 25% of patients who are diagnosed from incidental abnormal laboratory findings. Pharmacological therapies are useful in slowing disease progression and improving liver function tests; however, for many people liver transplantation is the only viable treatment for end-stage liver disease. This article provides an overview of the hypothesized aetiological factors, disease presentation, diagnosis and current treatment options.