primary biliary cholangitis

Question 1

typically seen in

Answer 1

middle ages females

Question 2

what happens

Answer 2

interlobular bile ducts become damaged by a chronic inflammatory process

causing progressive cholestasis, which may progress to cirrhosis

Question 3

classic presentation

Answer 3

itching in a middle aged woman

Question 4

assocs

Answer 4

sjogren’s
rheumatoid arthritis
systemic sclerosis
thyroid disease

Question 5

clinical features

Answer 5

fatigue
prutitis
cholestatic jaundice
hyperpigmentation 
clubbing 
hepatosplenomegaly

Question 6

Dx - immunology

Answer 6

anti-mitochondrial antibodies M2 subtype
smooth muscle antibodies
raised IgM

Question 7

Dx- imaging

Answer 7

required before diagnosis to exclude extrahepatic biliary obstruction

usually RUQ USS or MRCP

Question 8

Mx - 1st line

Answer 8

ursodeoxycholic acid

Question 9

Mx for itching

Answer 9

cholestyramine

Question 10

complications

Answer 10

cirrhosis –> PRTL HTN –> ascites, varices
otseomalacia, osteoporosis
hepatocellular carcinoma