Primary Biliary Cholngitis

Question 1

What is it?

Answer 1

Condition where intrahepatic or extrahepatic ducts become structured and fibrotic, causing an obstruction to bile flow out of liver and into intestines

Question 2

Cholangitis is?

Answer 2

Inflammation of the bile ducts

Question 3

Established association with?

Answer 3

Ulcerative colitis

Question 4

Presentation?

Answer 4

Jaundice 
Chronic right upper quadrant pain 
Pruritus 
Fatigue 
Hepatomegaly

Question 5

Diagnosis?

Answer 5

LFT: ALP is the most deranged, possible rise in bilirubin
MRCP: gold standard (magnetic resonance cholangiopancreatography, this involves MRI scan of liver, bile ducts and pancreas)

Question 6

Management?

Answer 6

Liver transplant
ERCP: to dilate and stent any strictures
Ursodeoxycholic acid to slow progression
Cholestyramine is bile acid sequestrate
Monitoring for complications

Question 7

ERCP?

Answer 7

Endoscopic retrograde cholangio-pancreatography
Involves inserting endoscope through oesophagus, stomach and duodenum to point where bile ducts empty into GI tract. Then go onto ampulla of vater and there they can enter bile ducts and inject contrast and use X rays to identify strictures which can be then dilated and stented