Primary biliary cholangitis Flashcards
1
Q
Primary biliary cholangitis definition
A
- chronic disease of small intrahepatic bile ducts
- progressive bile duct damage + chronic portal tract inflammation
2
Q
Primary biliary cholangitis risk factors
A
- more common in females (10:1)
- over 45 years old
- autoimmune history or family history
3
Q
Primary biliary cholangitis pathophysiology
A
- high antimitochondrial autoantibodies
- causes damage and destruction of biliary epithelial cells
- chronic portal tract inflammation leads to bile stasis, fibrosis and cirrhosis
4
Q
Primary biliary cholangitis signs and symptoms
A
- pruritus
- jaundice
- abdominal pain
- fatigue
- weight loss
- fever
- steatorrhoea
- splenomegaly
- ascites
- encephalopathy
5
Q
Primary biliary cholangitis investigations
A
- LFTs show cholestatic picture
- autoantibodies
- MRCP and liver biopsy
6
Q
Primary biliary cholangitis diagnosis
A
- cholestatic LFTs
- autoantibodies - AMA or ANA
- liver histology - classic bile duct lesions, portal tract inflammation, granuloma formation
7
Q
Primary biliary cholangitis management
A
- ursodeoxycholic acid (bile duct analogue, reduces cholesterol saturation of bile)
- obeticholic acid
- for pruritus - cholestyramine, naltrexone, rifampicin
