75. PSC and PBC

Question 1

What is primary biliary cholangitis?

Answer 1

Intralobular bile duct damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension

Question 2

What causes PBC?

Answer 2

Unknown environmental triggers, genetic predisposition

Question 3

How do patients with PBC present?

Answer 3

Lethargy, sleepiness and pruritus
Jaundice
Skin pigmentation 
Xanthelasma
Helatosplenomagaly

Question 4

What tests can be done for PBC?

Answer 4

LFT’S
ANTIMICHOMDRIAL ANTIBODIES! (Important)
Increased IgM

USS to rule out extrahepatic cholestasis
Biopsy can confirm granulomas (not usually needed)

Question 5

How is PBC treated?

Answer 5

Colestyramine/ naltrexone and rifampicin May help itching

Codeine phosphate- diorrhea

Osteoporosis prevention

Fat soluble vitamin supplements

Liver transplantation for end stage

Question 6

What is primary sclerosing cholangitis?

Answer 6

Progressive cholestasis with bile duct inflammation and strictures

Question 7

What are the signs of PSC?

Answer 7

Pruritus and fatigue
Ascending cholangitis
Cirrhosis
Hepatic failure

Question 8

Who is disposed to PSC?

Answer 8

Men
HLA-A1, B8, DR3 genes
IBD, (UC)

Question 9

How do you test for PSC?

Answer 9

ALP and bilirubin raised
Hypergammaglobuminaemia
Raised IgM
AMA-ve, ANA, SMA,ANCA may be positive

Question 10

How do you treat PSC?

Answer 10

Liver transplant for end stage disease

Yearly colonoscopy and USS as there’s such a high gallbladder,liver and colon cancer risk

Ursodeoxycholic acid got improve LFT’s

Colestyramine for pruritus (naltrexone and rifampicin May also help)