Primary Biliary Cirrhosis

Question 1

Pathology

Answer 1

Primary biliary cirrhosis is a condition where the immune system attacks the small bile ducts within the liver.

Primary biliary cirrhosis is a condition caused by chronic AUTOIMMUNE GRANULOMATOUS INFLAMMATION leading to bile duct damage

Question 2

Clinical Features

Answer 2

Fatigue

Pruritus

GI disturbance and abdominal pain

Jaundice

Pale stools

Xanthoma and xanthelasma

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

Question 3

Associations

Answer 3

Middle aged women

Other autoimmune diseases (e.g. thyroid, coeliac)

Rheumatoid conditions (e.g. systemic sclerosis, Sjogrens and rheumatoid arthritis)

Question 4

Management

Answer 4

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 5

Disease Progression

Answer 5

The most important end results of the disease are advanced liver cirrhosis and portal hypertension.

Some other issues / complications:

• Symptomatic pruritus
• Fatigue
• Steatorrhoea (greasy stools due to lack of bile salts to digest fats)
• Distal renal tubular acidosis
• Hypothyroidism
• Osteoporosis
• Hepatocellular carcinoma

Question 6

Diagnosis

Answer 6

LFTs show a cholestatic pattern: with a raised ALP and gamma-GT

The disease is characterised by the presence of ANTI-MITOCHONDRIAL ANTIBODIES

Question 7

Risk Factors

Answer 7

strong female preponderance

typical age at presentation is around 45-50 years old