Primary Sclerosing Cholangitis Flashcards
What is primary sclerosing cholangitis?
a condition where the intrahepatic and extrahepatic bile ducts become inflamed and damaged, developing strictures that obstruct the flow of bile out of the liver and into the intestines
What are the consequences of chronic bile obstruction? (3)
Hepatitis
Fibrosis
Cirrhosis
What is primary sclerosing cholangitis strongly linked with?
UC
What are the key risk factors of primary sclerosing cholangitis? (4)
• Male
• Aged 30-40
• Ulcerative colitis
• Family history
What are some features of primary sclerosing cholangitis? (6)
• Abdominal pain in the right upper quadrant
• Pruritus (itching)
• Fatigue
• Jaundice
• Hepatomegaly
• Splenomegaly
What is seen on LFTs in primary sclerosing cholangitis?
Raised ALP
Bilirubin raised later in disease
What is the diagnostic imaging in primary sclerosing cholangitis?
MRCP
Shows bile strictures
Why would colonoscopy be performed in primary sclerosing cholangitis?
To assess for UC
What can be used to treat strictures in primary sclerosing cholangitis?
ERCP
Structures are dilated
Stents can be put in
Antibiotics are given at same time to reduce risk of infection from ERCP
How is an ERCP performed?
This involves inserting an endoscope down the oesophagus, past the stomach, to the duodenum and the opening of the common bile duct (the sphincter of Oddi). This gives the operator access to the biliary system
What is the management of advanced primary sclerosing cholangitis?
Liver transplant
What is used to manage symptoms of Pruritis in primary sclerosing cholangitis?
Colestyramine
What does colestyramine do?
a bile acid sequestrant that reduces intestinal absorption of bile acids
Used to manage Pruritis
What are some complications of primary sclerosing cholangitis? (7)
• Biliary strictures
• Acute bacterial cholangitis
• Cholangiocarcinoma develops in 10-20% of cases
• Cirrhosis and the related complications (e.g., portal hypertension and oesophageal varices)
• Fat-soluble vitamin deficiency (A, D, E and K)
• Osteoporosis
• Colorectal cancer in patients with ulcerative colitis
What cancer is linked to primary sclerosing cholangitis?
Cholangiocarcinoma