Primary Biliary Cholangitis

Question 1

Primary biliary cholangitis (or primary biliary cirrhosis) is a chronic _______________ disease which develops due to progressive destruction of small and intermediate _______________ within the liver, subsequently evolving to ___________ and ______________.

Answer 1

cholestatic

bile ducts

fibrosis

cirrhosis

Question 2

Is PBC more common in men or women?

Answer 2

Women (ratio 9:1)

Question 3

What is the classic presentation of PBC?

Answer 3

Itching in a middle-aged woman

Question 4

What are the diseases associated with PBC? (4)

Answer 4

1. Sjogren’s (seen in up to 80% of patients)
2. RA
3. Systemic sclerosis
4. Thyroid disease

Question 5

What are the clinical features of PBC? (8)

Answer 5

1. Early - may be asymptomatic or fatigue, pruritus (+ raised ALP)
2. Cholestatic jaundice
3. Hyperpigmentation, especially over pressure points
4. Around 10% of patients have right upper quadrant pain
5. Xanthelasmas, xanthomas
6. Clubbing
7. Hepatosplenomegaly
8. Late: may progress to liver failure

Question 6

What would you expect to see in the LFTs of a patient with PBC (early)?

Answer 6

Elevated ALP (cholestatic picture)

Vs late, all LFTs may be more deranged if liver failure

Question 7

Which antibodies are associated with PBC?

Answer 7

Anti-mitochondrial (AMA) M2 subtype

Raised serum IgM

(Smooth muscle abs in 30% of patients)

(PBC = 3 M’s: mitochondria M2, IgM, middle-aged women)

Question 8

What is the first-line management of patients with PBC?

Answer 8

Ursodeoxycholic acid; slows disease progression and improves symptoms

Also recommended for patients with asymptomatic disease

Liver transplant in patients with advanced disease

Question 9

Primary sclerosing cholangitis is a biliary disease of unknown aetiology characterised by inflammation and fibrosis of _____________ hepatic bile ducts

Answer 9

intra AND extra-

Vs PBC which is intra-hepatic only

Question 10

What diseases are associated with PSC? (3)

Answer 10

1. UC (80% of patients with PSC have UC)
2. Crohn’s (much less than UC)
3. HIV

Question 11

What are the clinical features of PSC? (3)

Answer 11

1. Cholestasis (jaundice, pruritus, raised bilirubin and ALP)
2. RUQ pain
3. Fatigue

Question 12

What is the investigation of choice for suspected PSC?

Answer 12

ERCP or MRCP showing multiple biliary strictures giving a ‘beaded’ appearance

Question 13

What markers do you expect to find on the ANAs of a patient with PSC?

Answer 13

P-ANCA positive