Primary biliary cirrhosis

Question 1

What is primary biliary cirrhosis?

Answer 1

Autoimmune condition attacking the small bile ducts in the liver

Causing obstruction to bile outflow (cholestasis)

Back pressure leads to fibrosis, cirrhosis and liver failure ultimately

Question 2

Presentation of PBC

Answer 2

Fatigue

Pruritus (due to build up of bile acids)

GI disturbance and abdominal pain

Jaundice

Pale stools - pancreatic enzymes cannot reach bowel, build-up of bilirubin in the stool

Xanthoma and xanthelasma

Signs of cirrhosis and failure (e.g. ascites, splenomegaly, spider naevi)

Question 3

PBC is associated with

Answer 3

Middle aged women

Other autoimmune conditions e.g. thyroid, coeliac

Rheumatoid conditions e.g. systemic sclerosis, Sjogren’s and RA

Question 4

Diagnosis and investigations in suspected PBC

Answer 4

LFTs -

• ALP raised (tends to be first raised)
• Other liver enzymes and bilirubin may be raised in later disease

Autoantibodies:

• Anti-mitochondrial antibodies (most specific to PBC)
• Antinuclear antibodies (may also be present - 35%)

ESR raised
IgM raised

Liver biopsy is used to diagnose and stage the disease

FBC, U&Es, clotting to rule out other pathology

Question 5

Treatment of PBC

Answer 5

Ursodeoxycholic acid reduces the intestinal absorption of cholesterol

Colestyramine is a bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids

Liver transplant in end stage liver disease

Immunosuppression (e.g. with steroids) is considered in some patients

Question 6

Potential complications of PBC

Answer 6

Most important end results:

• Liver cirrhosis
• Portal hypertension

Other complications:

• Steatorrhoea
• Distal renal tubular acidosis
• Hypothyroidism
• Osteoporosis
• Hepatocellular carcinoma

Question 7

What autoantibodies are associated with PBC?

Answer 7

Anti-mitochondrial antibodies (most specific to PBC)

Antinuclear antibodies (may also be present - 35%)