Primary Biliary Cholangitis Flashcards
define primary biliary cholangitis?
chronic disease of small intrahepatic bile ducts-> characterised by progressive bile duct damage ( and eventual loss) occurring in context of chronic portal tract inflammation
fibrosis develops as consequence of original insulit
secondary effects of bile acids retained in liver=> CIRRHOSIS
outline the risk factors for primary biliary cholangitis?
female sex
age between 45-60 years
family history of PBC/autoimmune disease
smoking
outline the aetiology of PBC?
unknown
likely to be autoimmune
characterised by chronic autoimmune granulomatous inflammation-> leads to fibrosis, cirrhosis and portal, hypertension
genetic and environmental factors involved
An environmental trigger may cause bile duct epithelial injury, which then leads to a T-cell mediated autoimmune response directed against bile duct epithelial cells
summarise the epidemiology of PBC?
mainly affects middle aged women ( 9:1 female: male)
what are the presenting symptoms of PBC?
Fatigue
weight loss
pruritis
family history of autoimmune disease
history of hypercholestralaemia - cholestasis
May present with a complication of liver decompensation (e.g. jaundice, ascites, variceal haemorrhage)
May present with symptoms of associated conditions (e.g. Sjogren’s syndrome, arthritis, Raynaud’s phenomenon)
postural dizziness/ blackouts
what are the signs of PBC on physical examination
- Jaundice
- Skin pigmentation
- Scratch marks
- Xanthomas (secondary to hypercholesterolaemia which is a feature of cholestasis)
- Hepatosplenomegaly
- Ascites
- Signs of chronic liver disease
what are the appropriate investigations for PBC?
bloods
ultrasound
liver biopsy
describe bloods in PBC?
LFT
- High ALP and GGT
- bilirubin may be high or normal
- ALT and AST are normal initially-> but will increase as disease progresses and cirrhosis develops
- low albumin
- high prothrombin time
clotting
- prolongation of pT
typical features of PBC
- antimitochondrial antibodies ( typical feature of PBC) - AMAs are hallmarks of PBC
- high IgM
- high cholesterol
summarise typical presentation of PBC?
lethargy and pruritus female with minor increase in AST/gamma-GT and increase of alkaline phosphatase; hypercholesterolaemia, can be associated with Sjogren’s, Rheumatoid arthritis etc
what are the typical features of PBC?
▪ Antimitochondrial antibodies (typical feature of PBC) – AMAs are the hallmarks of PBC!!
▪ High IgM
High Cholesterol
outline the treatment for PBC?
Once diagnosis made treatment is important to prevent progression, with ursodeoxycholic acid (this is just exogenous bile salts).
Cholestyramine-> alleviate pruritus but must be given at least 2 hours apart from ursodeoxycholic acid.
patient may ultimately need transplantation
SIDE NOTE: Statins are not generally used in PBC.
Ursodeoxycholic acid lowers lipids and statins can obviously affect liver function therefore they do not form part of the initial treament.
what is used instead of statins in PBC?
ursodeoxycholic acid-> lowers lipids
statins can affect liver function-> don’t form part of initial treatment
why is an ultrasound a useful investigation for PBC
exclude extrinsicallyahepatic biliary obstruction ( gallstones)
What do we look for on a liver biopsy in PBC?
show chronic inflammatory cells and granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis and regenerating nodules of hepatocytes
