Both are autoimmune disease where the the bile ducts are affected.
Primary Sclerosing Cholangitis
- Intra and extra hepatic bile ducts are inflamed and damaged
- Strictures develop and sclerosis occurs
- Chronic bile obstruction leads to Hepatitis, fibrosis and Cirrhosis
- Strong association with Ulcerative Colitis
- Autoantibodies are not useful in diagnosis or assessment. None are sensitive or specific
- Stent on ERCP for treatment
Primary Biliary Cholangitis
- Intrahepatic bile ducts only
- Inflammation to epithelial cells of bile ducts can cause obstruction - cholestasis
- **Anti-mitochondrial antibodies (AMA)**are most specific and are part of diagnostic criteria
- Treatment with ursodeoxycholic acid- its a bile acid that makes the bile less harmful to the epithelial cells