Primary Sclerosing Cholangitis

Question 1

Primary sclerosing cholangitis

Answer 1

autoimmune
fibrosis and inflammation of intrahepatic and extra hepatic ducts. tightening of the ducts where there are fibrosis and dilation in others.

causes obstruction of bile out of the liver and into the intestines.

‘beaded appearance of the bile duct’ (concentric rings of fibrosis) “onion skin fibrosis”

Question 2

PSC pathophysiology

Answer 2

associated with ulcerative colitis (HLA)
HLA D8, HLA DR3
elevated IgM antibodies
p-ANCA*** (strong associated with IBD)

T cells attack the biliary cells

Question 3

PSC pathophysiology continued

Answer 3

similar to obstructive jaundice (beads obstruct bile)

epithelial cells die off= bile leaks into the interstitial space= pruritis, bile salt and acid in the skin. elevated conjugated bilirubin levels. raised ALP and GGT.

bilirubinuria (dark urine)
do not produce urobilinogen (not in urine) because it’s not been able to get to the gut.

Ix: ERCP

can cause complications involving the liver. fibrosis around the bile duct can constrict the portal veins (portal hypertension)

higher risk of cholangiocarcinoma

Question 4

primary sclerosing cholangitis risk factors and symptoms

Answer 4

Risk Factors
Male
Aged 30-40
Ulcerative Colitis
Family History

Presentation
Jaundice
Chronic right upper quadrant pain
Pruritus
Fatigue
Hepatomegaly

Question 5

Investigation for PSC

Answer 5

1. bloods: LFT shows a cholestatic picture (ALP)
2. autoantibodies (pANCA, ANA, ACL)
3. MRCP is gold standard.

Question 6

PSC management

Answer 6

liver transplant- curative

ERCP- dilate and stent any strictures.

Ursodeoxycholic acid is used and may slow disease progression

Colestyramine is a bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids

Monitoring for complications (such as cholangiocarcinoma, cirrhosis and oesophageal varices)

Question 7

what is an ERCP?

Answer 7

(Endoscopic Retrograde Cholangio-Pancreatography)

Inserting a camera through the persons throat, oesophagus, stomach and duodenum to the a point in the duodenum where the bile ducts empty into the GI tract.

They then go through the sphincter of Oddi and into the ampulla of Vater. From the ampulla of Vater they can enter into the bile ducts and use X-rays and injecting contrast to identify any strictures.

These strictures can then be dilated and stented during the same procedure providing improved flow through those ducts and an improvement in symptoms.