Has many features to suggest that it is an autoimmune disease, the system that drains bile from the liver into the intestines is progressively destroyed, the liver becomes scarred, leading to cirrhosis.
Blood tests strongly suggest the correct diagnosis, but a liver biopsy is needed for confirmation. It is also usually necessary to x ray the biliary system to look for other causes of obstruction.
Ursodeoxycholic acid, a chemical that dissolves gall stones, provides substantial symptomatic relief. It is still unclear if it slows liver damage. Patients also do well with liver transplantation.