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.
Sargent
Suzanne
S
Institute of Liver Studies, King's College Hospital, London.
Fullwood
Danielle
D
D018450:Disease Progression; D005221:Fatigue / Q000209:etiology; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology