Primary biliary cholangitis & primary sclerosing cholangitis Flashcards
Describe Primary biliary cholangitis (PBC)?
- Chronic, progressive cholestatic liver disease
- Women > 30 years
- Associated with AMA
- Inflammation of the portal tracts, leading to loss of smaller bile ducts
- Leads to fibrosis and cirrhosis of the liver
Describe the pathophysiology of PBC?
- Immune mechanisms
- Associated with other AI diseases
- HLA-DR8
- Anti-mitochondrial antibodies (AMA)
- Elevated IgM
What are the clinical features of PBC?
- Pruritis
- Worse on limbs
- Fever, rigors
- Bone pain, fractures (fat-soluble vitamin malabsorption)
- Jaundice occurs late in disease progression
What are the associated diseases of PBC?
- Sicca syndrome
- Systemic sclerosis
- Coeliac disease
- Thyroid diseases
Describe the diagnosis and investigations into PBC?
- Cholestasis pattern on LFTs
- Hypercholesterolaemia
- AMA positive in majority
- ANA, anti-smooth muscle antibodies can also be present
- MRCP to exclude other biliary diseases
- US shows no sign of biliary obstruction
- Liver biopsy if diagnosis uncertain
Differentials for PBC?
- Obstructive bile duct lesion
- Autoantibodies are negative
- US shows duct dilatation
- Small-duct primary sclerosing cholangitis
- More likely to be younger males
Describe the management of PBC?
- Treat side effects
- Pruritis, malabsorption, bone disease
- Ursodeoxycholic acid
- Prednisolone
- Liver transplantation
- Should be considered once liver failure has developed
Describe the use of ursodeoxycholic acid in the management of PBC?
- Replaces toxic hydrophobic bile acids
- Reduces apoptosis of the biliary epithelium
- May cause nausea or weight gain
What is the most reliable marker of decling liver function?
Bilirubin
Describe the management of pruritis secondary to PBC?
Anion-biding resin colestyramine
Describe the management of malabsorption secondary to PBC?
Replacement of fat soluble vitamins
Describe the management of bone disease secondary to PBC?
- Calcium and vitamin D3 replacement
- Bisphosphonates if evidence of osteoporosis
Describe Primary sclerosing cholangitis (PSC)?
- Cholestatic liver disease caused by diffuse inflammation and fibrosis
- Leads to obliteration of intrahepatic and extrahepatic bile ducts
- => biliary cirrhosis, portal HTN, hepatic failure
- Cholangiocarcinoma develops in some
- More common in young men
Describe the general diagnostic criteria for PSC?
- Generalised beading and stenosis of the biliary system on cholangiography
- Absence of choledoscholithiasis (or history of bile duct surgery)
- Exclusion of bile duct cancer, by prolonged follow up
What does Secondary sclerosing cholangitis mean?
Changes of PSC when a clear predisposing factor for duct fibrosis can be identified
What diseases are associated w ith PSC?
