GI - Primary Biliary Cholangitis and Pimary sclerosing cholangitis

Question 1

What is primary Biliary cholangitis? What is the classic presentation?

Answer 1

• Previously referred to as primary biliary cirrhosis
• chronic liver disorder, usually sene in middle-aged females (9:1)
• Aetiology not fully understood but is auto-immune condition - interlobular bile ducts become damaged by chronic inflammatory process, which causes progressive cholestasis, which may eventually progress to cirrhosis
• Classic presentation: itching muddled-aged female

Question 2

What are the clinical features of primary biliary cholangitis?

Answer 2

• Early: may be asymptomatic (eg raised ALP on routine LFTs) or fatigue/pruritus
• Late: may progress to liver failure
• Cholestatic jaundice
• Hyperpigmentation: especially over pressure points
• Approx 10% have RUQ pain
• May have: xanthelasmas, xanthomas, clubbing, hepatosplenomegaly

Question 3

What are complications of primary biliary cholangitis?

Answer 3

• Malabsorption: osteomalacia/coagulopathy
• Sicca syndrome: 70% of cases (eg xerostomia)
• Portal hypertension: Ascites, variceal haemorrhage
• Hepatocellular carcinoma: 20 fold increased risk

Question 4

What diseases are associated with primary biliary cholangitis?

Answer 4

• Sjögren’s syndrome (seen in 80% of pts)
• RA
• systemic sclerosis
• Thyroid disease

Question 5

How do you diagnose primary biliary cholangitis?

Answer 5

-Anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of pts and are highly specific
-anti-SM (smooth muscle) antibodies in 30% of pts
-Raised serum IgM
(rule of M’s)

Question 6

What is the management for primary biliary cholangitis?

Answer 6

• Pruritus: cholestyramine
• Fat-soluble vitamin supplementation
• ursodeoxycholic acid
• Life transplant (esp if bilirubin becomes v high)

Question 7

What is primary sclerosing cholangitis?

Answer 7

-Biliary Disease of unknown aetiology - characterised by inflammation and fibrosis of intra and extra hepatic bile ducts

Question 8

What diseases are associated with primary sclerosing cholangitis?

Answer 8

• Ulcerative colitis: 4% of pts with UC have PSC, 80% of pts with PSC have UC
• Crohn’s (much less common association than UC)
• HIV

Question 9

What are the features of primary sclerosing cholangitis?

Answer 9

• Cholestasis: jaundice and pruritus
• RUQ pain
• fatigue

Question 10

What Ix would you do for primary sclerosing cholangitis?

Answer 10

• ERCP: standard diagnostic tool - shows multiple biliary strictures giving a ‘beaded’ appearance
• ANCA may be positive
• Biopsy (limited role): may show fibrous obliterative cholangitis often described as ‘onion skin’

Question 11

What are important complications of primary sclerosing cholangitis?

Answer 11

• Cholangiocarcinoma in 10% of pts

- Increased risk of colorectal cancer