Primary sclerosing cholangitis

Question 1

Primary sclerosing cholangitis is the chronic progressive cholestatic liver disease due to inflammation, fibrosis and stricturing of intrahepatic and/or extrahepatic bile ducts.

Eventual progression to liver cirrhosis, portal hypertension and liver failure.

Occurs more in males (3:1)
Usually 30-40 years old
Strong association with IBD (UC)

Question 2

Pathophysiology of PSC

Answer 2

1. Genetics - HLA-DRB1
2. Immune dysregulation and autoantibodies (pANCA)
3. Altered bile acid composition and signaling causes biliary injury, inflammation and fibrosis

Question 3

Clinical presentation of patients with PSC

Answer 3

1. Male in 30-40 years old
2. Incidental elevated ALP, GGT
3. Non-specific symptoms
   - Fatigue, weight loss, RUQ pain
   - Jaundice and pruritus
   - Recurrent bacterial cholangitis
4. Concomitant IBD
   - Abdominal pain, diarrhoea, rectal bleeding
5. Advanced liver disease

Question 4

Diagnostic investigations for PSC

Answer 4

1. LFT - elevated ALP, GGT, hyperbilirubinaemia
   (ALT, AST mildly elevated)
2. Serum IgG4 levels - TRO IgG4 associated cholangitis
3. MRCP - multifocal strictures and segmental dilation of intrahepatic and extrahepatic bile ducts
   (beaded or pruned tree appearance)
4. Liver biopsy - onion skin fibrosis
5. ERCP if MRPC is inconclusive

Question 5

Complications of PSC

Answer 5

1. Severe stricture
2. Bacterial cholangitis
3. Cholangiocarcinoma
4. Gallbladder polyps and cancer
5. Colorectal cancer
6. Liver cirrhosis
7. Osteoporosis
8. Fat soluble vitamin deficiency

Question 6

Management of PSC

Answer 6

No curative therapy

1. Ursodeoxycholic acid
2. Bile acid sequestrants (cholestyramine, colestipol)
3. Fat soluble vitamin supplement
4. Calcium and vitamin D supplement
5. ERCP dilatation
6. Regular surveillance with MRI/MRCP, colonoscopy for malignancy
7. Liver transplant