3.7. Liver + Biliary Tree Disease - Primary Sclerosing Cholangitis

Question 1

What type of disease is Primary Sclerosing Cholangitis?

Answer 1

An autoimmune disease

Question 2

What other Bowel Disease is Primary Sclerosing Cholangitis related to?

Answer 2

Inflammatory Bowel in Disease 75% of cases - Usually Ulcerative Colitis

Question 3

What else can Primary Sclerosing Cholangitis be related to?

Answer 3

An increase in Serum Antineutrophil Cytoplasmic Antibodies (Serum ANCA)

Question 4

Is Primary Sclerosing Cholangitis more common in Males or Females?

Answer 4

Males

Question 5

What is the Pathology of Primary Sclerosing Cholangitis?

Answer 5

Auto-antibodies result in the Fibrosing Inflammatory Destruction of Bile Ducts

Question 6

Are Intrahepatic Bile Ducts, Extrahepatic Bile Ducts, of Both Effected?

Answer 6

Both

Question 7

What does the Fibrosis cause?

Answer 7

Stricture of the Bile Ducts

Question 8

What are the Clinical Signs of Primary Sclerosing Cholangitis?

Answer 8

1. Increased Serum Antineutrophil Cytoplasmic Antibodies (ANCA)
2. Increased Alkaline Phosphatase (ALP)

Question 9

Why is there a raised Serum Alkaline Phosphatase?

Answer 9

This is present in Hepatic Canalicular and Sinusoidal Membranes, and is released into the Blood in Intra/Extrahepatic causes of Cholestatic Disease due to an increase in its synthesis

Question 10

What are the Symptoms of Primary Sclerosing Cholangitis?

Answer 10

1. Jaundice
2. Recurrent Cholangitis
3. Inflammatory Bowel Disease Symptoms
4. Steatorrhoea
5. Vitamin Deficiency

Question 11

What type of Jaundice Occurs?

Answer 11

Post-Hepatic Jaundice (mainly)
Intrahepatic Jaundice (for the Intrahepatic Ducts which are effected)

Question 12

What is Recurrent Cholangitis? And why does it occur?

Answer 12

This is the Swelling associated with the Chronic Inflammation

Question 13

Why is Inflammatory Bowel Disease Symptoms associated with this?

Answer 13

Due to the massive overlap of the patients who present with both the diseases

Question 14

Why might Steatorrhoea / Vitamin Deficiencies occur?

Answer 14

Due to the decrease in Bile, The Small intestines cannot properly absorb fat and so this will be excreted

Question 15

What investigations are required for Primary Sclerosing Cholangitis?

Answer 15

1. Magnetic Resonance Cholangio-Pancreatography (MRCP) with Biopsy
2. Liver Function Tests (and Biochemistry)

Question 16

What is the test of choice?

Answer 16

Magnetic Resonance Cholangio-Pancreatography (MRCP)

Question 17

What is the purpose of Magnetic Resonance Cholangio-Pancreatography (MRCP)?

Answer 17

To view the damage (fibrosis) of the Biliary Tree

Question 18

What is the Liver Function Test (and Biochemistry) looking for in Primary Sclerosing Cholangitis?

Answer 18

A raised Alkaline Phosphatase

Question 19

What are the treatment options for Primary Sclerosing Cholangitis?

Answer 19

1. Transplantation

2. Biliary Stenting

Question 20

Why is transplantation necessary?

Answer 20

Due to there being no cure for this disease, this is the only Long-Term Alternative

Question 21

What is the Purpose of Biliary Stenting?

Answer 21

This is used to relieve the Symptoms which are caused by a blockage of bile

Question 22

What can Primary Sclerosing Cholangitis lead to?

Answer 22

1. Further Cirrhosis of the Liver

2. Cholangio Carcinoma