primary sclerosis cholangitis Flashcards
what is PSC?
Primary sclerosing cholangitis is a condition where the intrahepatic or extrahepatic ducts become strictured and fibrotic.
This causes an obstruction to the flow of bile out of the liver and into the intestines. Sclerosis refers to the stiffening and hardening of the bile ducts, and cholangitis is inflammation of the bile ducts.
Chronic bile obstruction eventually leads to liver inflammation (hepatitis), fibrosis and cirrhosis.
what is PSC associated with?
There is an established association with ulcerative colitis, with around 70% of cases being alongside established ulcerative colitis.
what will the bloods look like?
cholestatic picture with an increase in ALP
what is the role of antibodies in diagnosing PSC?
No antibodies are highly sensitive or specific to PSC. They aren’t very helpful in diagnosis but they can indicate where there is an autoimmune element to the disease that may respond to immunosuppression.
- > Antineutrophil cytoplasmic antibody (p-ANCA) in up to 94%
- > Antinuclear antibodies (ANA) in up to 77%
- > Anticardiolipin antibodies (aCL) in up to 63%
what is the gold standard for diagnosing PSC?
magnetic resonance cholangiopancreatography. This involves an MRI scan of the liver, bile ducts and pancreas.
In primary sclerosis cholangitis the ducts have a beaded appearance
what is the management of PSC?
- Liver transplant can be curative but is associated with its own problems (around 80% survival at 5 years).
- ERCP can be used to dilate and stent any strictures
- Colestyramine is a bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids
- Monitoring for complications (such as cholangiocarcinoma, cirrhosis and oesophageal varices)
what are complications of PSC?
- Acute bacterial cholangitis
- Cholangiocarcinoma develops in 10-20% of cases
- Colorectal cancer
- Cirrhosis and liver failure
- Biliary strictures
- Fat soluble vitamin deficiencies (AEDK)
