Primary Sclerosing Cholangitis

Question 1

Description: What is primary sclerosing cholangitis?

Answer 1

• Autoimmune chronic liver disease in which BOTH INTRAHEPATIC AND EXTRAHEPATIC bile ducts are slowly destroyed and inflamed resulting in biliary STRICTURES, affecting mainly males

Question 2

Aetiology/Risk Factors:

Answer 2

XXX

Question 3

Pathology: Describe the inflammation in primary sclerosing cholangitis and what this leads to (3)

Answer 3

• Chronic inflammation and fibrous obliteration of bile ducts
• Caused loss of intrahepatic and extra hepatic bile ducts
• Progresses to cirrhosis

Question 4

Symptoms: What are the symptoms of primary sclerosing cholangitis?

Answer 4

• Jaundice
• Recurrent cholangitis
• Pruritis
• Abdominal pain

Question 5

Signs:

Answer 5

XXX

Question 6

Investigations: How do we investigate for primary sclerosing cholangitis?

Answer 6

• pANCA is positive

- MRCP (diagnostic)

Question 7

Treatment: How do we treat prmiary sclerosing cholangitis?

Answer 7

• Liver transplant

- Biliary stents

Question 8

Complications: What are the complications of primary sclerosing cholangitis?

Answer 8

• Cirrhosis

- Cholangiocarcinoma

Question 9

Extra: What disease is associated with primary sclerosing cholangitis?

Answer 9

Inflammatory Bowel Syndrome (i.e ulcerative colitis)

Question 10

Extra: What are you at an increased risk of developing if you have primary sclerosing cholangitis?

Answer 10

• Cholangiocarcinoma (malignancy of biliary tree)