Autoimmune Cholangitis

Question 1

What is PBC

Answer 1

Primary Biliary Cholangitis, intralobular bile ducts damaged by chronic autoimmune granulomatous inflammation

Question 2

Signs of PBC

Answer 2

Presents around 50yrs
Often asymptomatic with raised ALP incidental finding
Lethargy, sleepiness, pruritis for years before
Jaundice
Hepatosplenomegaly

Question 3

PBC complications

Answer 3

Cirrhosis complications

Malabsorption of Vit ADEK

Question 4

PBC tests

Answer 4

Inc ALP and GGT
AMA M2 subtype positive in antibody screen
US to exclude other causes

Question 5

PBC treatment

Answer 5

Symptomatic - colestyramine 4-8g/24h for pruritis, codeine 30mg/8h for diarrhoea
Vitamin prophylaxis (ADEK)
Liver transplant for end-stage disease or intractable pruritis

Question 6

What is PSC

Answer 6

Progressive cholestasis with bile duct inflammation + strictures

Question 7

PSC signs

Answer 7

Pruritis, fatigue

Advanced shows ascending cholangitis/ cirrhosis/ hepatic failure

Question 8

PSC associations

Answer 8

Associated with UC

Associated with HLA-A1/B8/DR3 genes

Question 9

PSC in cancer

Answer 9

Inc risk of bile duct/ gallbladder/ liver/ colon cancers so yearly colonoscopy + US

Question 10

PSC tests

Answer 10

Inc ALP + IgM
pANCA +ve
AMA -ve, ANCA/SMA/ANA +ve
ERCP/MRCP show duct damage

Question 11

PSC treatment

Answer 11

Ursodeoxycholic acid may improve LFTs
Antibiotics for bacterial cholangitis
Colestyramine 4-8g/24h for pruritis, naltrexone + rifampicin may help
Liver transplant for end-stage disease

Worse prognosis for those with IBD as 5-10% get colon cancer post-transplant