PBC + PSC

Question 1

PBC stands for?

Answer 1

primary biliary cholangitis

Question 2

What is PBC?

Answer 2

Intrahepatic autoimmune jaundice
Affecting interlobular bile ducts (not CBD itself)

Question 3

RF of PBC?

Answer 3

Female, 40-50 y/o
other autoimmune diseases
smoking

Question 4

MC or less common than PSC

Answer 4

PBC more common than PSC

Question 5

Pathology of PBC

Answer 5

AutoAb cause interlobular bile duct damage - CHRONIC AUTOIMMUNE GRANULOMATOUS INFLAMMATION

Result in cholestasis (blockage of bile) = fibrosis, cirrhosis, portal htn, infection

Question 6

Sx of PBC

Answer 6

Initially aSx, routine test shows High AMA (anti mitochondrial Abs)

Pruritus + fatigue earliest, then jaundice, then hepatomegaly + xanthelesma (yellow growth by corners of eyelids = cholesterol build up)

Question 7

What is pruritus?

Answer 7

Conj bilirubin excess can leak out + cause skin to itch

Question 8

Complications of PBC

Answer 8

Cirrhosis, malabsorption of fats + ADEK (Therefore steorrhoea), osteomalacia, coagulopathy

Question 9

Dx of PBC

Answer 9

LFT = High ALP, Conj bilirubin, low albumin

Rule our acute hep = HepBsAg, HCVAb

Serology = 95% have AMA Abs (esp M2) - specific
1st line = USS to exclude extra hepatic cholestasis
Liver biopsy = portal tract infiltrate (lymphocyte + plasma cell)
40% granulomatous
portal tract fibrosis

Question 10

Tx of PBC?

Answer 10

1st line (improves Sx) = Ursodeoxycholic Acid (lifelong)

For pruritus = Cholestyramine (relief itching)

Vit ADEK suppose consider osteomalacia
May ultimately need liver transplant (PBC = major indication for it)

Question 11

Why is Ursodeoxycholic acid used?

Answer 11

Bile acid analogue, dampens immune response and decrease cholestasis