Primary biliary cholangitis (PBC) is a chronic, immune-mediated cholestatic liver disease in which the small intrahepatic bile ducts are progressively destroyed. Cholestasis means impaired bile flow: losing those ducts impairs the flow of bile, and the retained bile acids damage the liver. Over years a proportion of patients develop cirrhosis (advanced scarring of the liver) and portal hypertension (raised pressure in the portal venous system that carries blood from the gut to the liver). PBC is the most common chronic cholestatic liver disease in adults, and it affects mainly middle-aged women.
What organises the topic is that the immune attack is aimed at the small bile ducts rather than at the liver as a whole, so the earliest changes are duct destruction and cholestasis. That is why the diagnosis usually rests on cholestatic liver tests and a characteristic autoantibody rather than on biopsy, and why treatment is judged by how completely the biochemistry responds.
Choose a route through this family
A reader new to PBC can follow the order of the disease itself: why it happens, how it presents, how it is recognised, how it is treated and what happens over time. A reader looking for one question can go straight to the matching note.
- Pathogenesis of Primary Biliary Cholangitis: the immune attack on the small bile ducts, the role of anti-mitochondrial antibodies, molecular mimicry and genetic susceptibility. It explains why the disease behaves as it does.
- Clinical Features of Primary Biliary Cholangitis: how PBC presents, from detection on routine liver tests to fatigue, pruritus and the autoimmune conditions that occur alongside it.
- Diagnosis of Primary Biliary Cholangitis: the diagnostic criteria, the autoantibody profile, the place of liver biopsy and the Ludwig staging of the histological changes.
- Treatment of Primary Biliary Cholangitis: how ursodeoxycholic acid and second-line drugs slow progression, how pruritus and the other symptoms are managed, and when liver transplantation is considered.
- Prognosis and Complications of Primary Biliary Cholangitis: the natural history of the disease, the progression to cirrhosis and portal hypertension, and the raised risk of hepatocellular carcinoma.