Primary biliary cholangitis

Question 1

[…] condition causing […] and […] of the […] […], eventually leading to liver […].

Answer 1

autoimmune condition causing scarring and inflammation of the bile ducts, eventually leading to liver cirrhosis.

Question 2

Epidemiology

Answer 2

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

Question 3

Features

Extreme […]
[…]
Dry skin
Dry eyes
[…]
Patients are at higher risk of developing […] […], and prognosis is poor.

Answer 3

Features

Extreme fatigue
Itching
Dry skin
Dry eyes
Jaundice
Patients are at higher risk of developing hepatocellular carcinoma, and prognosis is poor.

Question 4

PBC Key investigation

Positive […]-[…] antibodies, […] subtype
raised serum […]

Answer 4

Positive Anti-mitochondrial antibodies, M2 subtype
raised serum IgM

Question 5

Associations

[…] syndrome (seen in up to 80% of patients)
[…] arthritis
[…] […]
[…] disease

Answer 5

Associations

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

Question 6

Mx

1st line –> […] […]
pruritus: […]
[…]-[…] vitamin supplementation
Liver […]

Answer 6

ursodeoxycholic acid
pruritus: cholestyramine
fat-soluble vitamin supplementation
Liver transplant

Question 7

liver transplantation

e.g. if bilirubin > […] (PBC is a major indication)
recurrence in graft can occur but is not usually a problem

Answer 7

liver transplantation

e.g. if bilirubin > 100 (PBC is a major indication)
recurrence in graft can occur but is not usually a problem

Question 8

PBC complciations

[…] → portal […] → […], variceal […]
[…] and osteoporosis
significantly increased risk of […] […] (20-fold increased risk)

Answer 8

cirrhosis → portal hypertension → ascites, variceal haemorrhage
osteomalacia and osteoporosis
significantly increased risk of hepatocellular carcinoma (20-fold increased risk)