Primary biliary cirrhosis

Question 1

Another name

Answer 1

Cholangitis

Question 2

Most common patient

Answer 2

Women aged 40-50 (90% of patients)
Far more common in females
Typical age of presentation is 50

Question 3

Describe

Answer 3

A chronic disorder with progressive destruction of small bile ducts, leading to cirrhosis

Question 4

Aetiology

Answer 4

Unknown

But immunological and serum anti-mitochondrial antibodies (AMA) are found in almost all patients

Question 5

Risk factors

Answer 5

• Positive family history
• Many UTIs
• Smoking
• Past pregnancy
• Other autoimmune disease
• Use of nail polish/hair dye

Question 6

Pathophysiology

Answer 6

• Interlobar bile ducts are damaged by CHRONIC AUTOIMMUNE GRANULOMATOUS INFLAMMATION resulting in cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
• Serum anti-mitochondrial antibodies (AMA) found in almost all patients
• Likely that an environmental factor acts on genetically predisposed hosts
  to trigger disease

Question 7

Complications

Answer 7

• Cirrhosis
• Osteoporosis
• Malabsorption of fat-soluble vitamins (A,D,E,K) due to cholestasis
• Decreased bilirubin in the gut lumen results in osteomalacia and coagulopathy

Question 8

Clinical presentation

Answer 8

Asymptomatic patients are discovered on routine examination or screening and may have hepatomegaly, a raised serum alkaline phosphate or anti- mitochondrial antibodies (AMA)
Pruritus (itching) and jaundice after
Lethargy and fatigue
Pigmented xanthelasma (yellow fat deposits under skin usually around eyelids) on eyelids deposits of cholesterol in the creases of the hands may be seen

Question 9

What is earliest symptom

Answer 9

Pruritus (itching)

Question 10

Differential diagnosis

Answer 10

• Autoimmune cholangitis

- Extrahepatic biliary obstruction should be exclude by ultrasound

Question 11

Diagnosis

Answer 11

Blood
Ultrasound (can show alteration in liver architecture)
Liver failure shows characteristic histological features

Question 12

What would you look for on blood test

Answer 12

• Increased alkaline phosphate
• Raised serum cholesterol
• Anti-mitochondrial antibodies (AMAs) - present in 95%, M2 antibody is 98% specific
• Raised serum IgM

Question 13

Histological features of liver failure

Answer 13

• Portal tract infiltrate, mainly of lymphocytes and plasma cells
• Around 40% have granulomas
• Damage to and loss of small bile ducts and ductular proliferation
• Portal tract fibrosis and eventually cirrhosis is seen

Question 14

Treatment

Answer 14

• URSODEOXYCHOLIC ACID:
  Improves bilirubin and aminotransferase levels
• Supplementation of Vitamin A,D,E,K (fat-soluble vitamins)
• BISPHOSPHONATES for osteoporosis
• Due to lack of effective medical therapy, primary biliary cirrhosis is a major indication for LIVER TRANSPLANTATION

Question 15

Treatment of Pruritus

Answer 15

• COLESTYRAMINE works but unpalatable

* NALOXONE and NALTREXONE (opioid antagonists) shown to help