Primary sclerosing cholangitis Flashcards
1
Q
Primary sclerosing cholangitis definition
A
- chronic and progressive cholestatic liver disease
- inflammation and fibrosis of bile ducts causes stricture formation
2
Q
Primary sclerosing cholangitis risk factors
A
- more common in males (2:1)
- 75% have IBD, typically UC
- family history
3
Q
Primary sclerosing cholangitis pathophysiology
A
- 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
4
Q
Primary sclerosing cholangitis signs and symptoms
A
- abdominal pain
- pruritus
- fatigue
- weight loss
- fever
- jaundice
- steatorrhoea
- ascites
- splenomegaly
- encephalopathy
5
Q
Primary sclerosing cholangitis diagnosis
A
- LFTs may show cholestatic picture
- imaging is essential for diagnosis
- abdominal ultrasound, CT
- MRCP preferred as less invasive, ERCP can be therapeutic
6
Q
Primary sclerosing cholangitis management
A
- 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
