Primary sclerosing cholangitis

Question 1

Primary sclerosing cholangitis definition

Answer 1

• chronic and progressive cholestatic liver disease
• inflammation and fibrosis of bile ducts causes stricture formation

Question 2

Primary sclerosing cholangitis risk factors

Answer 2

• more common in males (2:1)
• 75% have IBD, typically UC
• family history

Question 3

Primary sclerosing cholangitis pathophysiology

Answer 3

• likely immune related
• inflammation and injury of medium to large bile ducts
• leads to strictures and fibrosis, causing bile stasis
• leads to stones and liver damage

Question 4

Primary sclerosing cholangitis signs and symptoms

Answer 4

• abdominal pain
• pruritus
• fatigue
• weight loss
• fever
• jaundice
• steatorrhoea
• ascites
• splenomegaly
• encephalopathy

Question 5

Primary sclerosing cholangitis diagnosis

Answer 5

• LFTs may show cholestatic picture
• imaging is essential for diagnosis
• abdominal ultrasound, CT
• MRCP preferred as less invasive, ERCP can be therapeutic

Question 6

Primary sclerosing cholangitis management

Answer 6

• conservative - lifestyle changes
• symptomatic management of pruritus and osteopenia (bile function impaired means low vit D absorption)
• ERCP and balloon dilation of stricture
• liver transplant in end stage disease