Primary biliary cholangitis

Question 1

Primary biliary cholangitis (previously referred to as primary biliary cirrhosis) is a chronic liver disorder typically seen in

Answer 1

middle-aged females (female:male ratio of 9:1)

Question 2

Pathophysio of primary billary cholangitis ?

Answer 2

Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis

Question 3

X

Question 4

Clinical features of primary billary cholangitis

Answer 4

fatigue, pruritus

cholestatic jaundice

hyperpigmentation, especially over pressure points

around 10% of patients have right upper quadrant pain

xanthelasmas, xanthomata

also: clubbing, hepatosplenomegaly

late: may progress to liver failure

Question 5

C

Question 6

X

Question 7

X

Question 8

Diagnosis ?

Answer 8

early: may be asymptomatic (e.g. raised ALP

Diagnosis
immunology
anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
smooth muscle antibodies in 30% of patients

raised serum IgM

imaging - right upper quadrant Us or MRCP
required before diagnosis to exclude an extrahepatic biliary obstruction

Question 9

X

Question 10

X

Question 11

Associating of primary billary cholangitis

Answer 11

Sjogren’s syndrome (seen in up to 80% of patients)
rheumatoid arthritis
systemic sclerosis
thyroid disease

Question 12

Mx of primary sclerosis g cholangitis

Answer 12

first-line: ursodeoxycholic acid
slows disease progression and improves symptoms
pruritus: cholestyramine
fat-soluble vitamin supplementation
liver transplantation
e.g. if bilirubin > 100 (PBC is a major indication)
recurrence in graft can occur but is not usually a problem

Question 13

X

Question 14

X

Question 15

X

Question 16

Complication

Answer 16

cirrhosis → portal hypertension → ascites, variceal haemorrhage

osteomalacia and osteoporosis

significantly increased risk of hepatocellular carcinoma (20-fold increased risk)