Primary biliary cholangitis (PBC) is a chronic, immune-mediated cholestatic liver disease in which the small intrahepatic bile ducts are progressively destroyed. Losing those ducts impairs the flow of bile, and the retained bile acids damage the liver; over years a proportion of patients develop cirrhosis and portal hypertension. 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
If PBC is new to you, start with why it happens and how it presents, then move to diagnosis and treatment:
- Pathogenesis of Primary Biliary Cholangitis: the immune attack on the small bile ducts, the role of anti-mitochondrial antibodies, molecular mimicry and genetic susceptibility.
- 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.