3.6. Liver + Biliary Tree Disease - Primary Biliary Cirrhosis

Question 1

What is the known Aetiology of Primary Biliary Cirrhosis?

Answer 1

Trick Question - It is Unknown

Question 2

What is the Suspected Aetiology of Primary Biliary Cirrhosis?

Answer 2

An autoimmune disorder

Question 3

Why is this thought to be an Autoimmune disorder?

Answer 3

Due to immunological mechanisms playing a part

Question 4

Is Primary Biliary Cirrhosis more common in Males or Females?

Answer 4

Females

Question 5

In what age group is Primary Biliary Cirrhosis most apparent?

Answer 5

40-50 years old

Question 6

What is the term used for an early, preceding lesion?

Answer 6

Chronic, Non-Suppurative, Destructive Cholangitis

Question 7

What does Non-Suppurative mean?

Answer 7

Without pus formation on inflammation

Question 8

What does the preceding lesion highlight?

Answer 8

That it is only Late-Stage which ends in Cirrhosis

Question 9

What is the Pathology of Primary Biliary Cirrhosis?

Answer 9

There is a progressive destruction of the Small Bile Ducts, eventually leading to Cirrhosis

Question 10

What is found in most patients with Primary Biliary Cirrhosis?

Answer 10

Serum Anti-Mitochondrial Antibodies (AMA positive disease)

Question 11

What causes the initiation of the Pathology of Primary Biliary Cirrhosis?

Answer 11

It is most likely that an Environmental Factor acts on a Genetically Predisposed Host - via Molecular Mimicry - initiating autoimmunity

Question 12

What does the Molecular Mimicry (initiating the Autoimmunity) cause?

Answer 12

Granulomatous Formation

Question 13

What does Granulomatous Formation result in?

Answer 13

Reduction in the effectiveness of the Intrahepatic Ducts, eventually leading to Death and Cirrhosis

Question 14

What are the Clinical Signs of Primary Biliary Cirrhosis?

Answer 14

1. Serum Anti-Mitochondrial Antibodies
2. A Raised Serum Alkaline Phosphatase (ALP)
3. Hepatomegaly
4. Xanthelasma (and other Cholesterol Deposits)

Question 15

Why is there raised Serum Alkaline Phosphatase (ALP)?

Answer 15

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 16

Why does Hepatomegaly occur?

Answer 16

Due to the damage and inflammation of the Liver

Question 17

What is Xanthelasma?

Answer 17

A Yellowish Fat Deposition around the Skin of the Eye

Question 18

Why does Xanthelasma occur (and other Cholesterol Deposits)?

Answer 18

Due to the Liver Inability to deal with Cholesterol any more

Question 19

What are the Symptoms of Primary Biliary Cirrhosis?

Answer 19

1. Asymptomatic (usually)
2. Pruritus
3. Jaundice
4. Fatigue (and other generalized symptoms)

Question 20

How is Primary Biliary Cirrhosis normally discovered, if Asymptomatic?

Answer 20

1. On a routine examination for something else

2. Screening

Question 21

What is Pruritus?

Answer 21

Itching

Question 22

Why does Pruritus occur in Primary Biliary Cirrhosis?

Answer 22

Due to the Elevated Levels of Bilirubin in the Blood

Question 23

What type of Jaundice will occur in Primary Biliary Cirrhosis?

Answer 23

Intrahepatic / Post-Hepatic Jaundice

Question 24

What investigations are required for Primary Biliary Cirrhosis?

Answer 24

1. Liver Function Test (and Biochemistry)
2. Serum Anti-Mitochondrial Antibody (AMA) test
3. Abdominal Ultrasound
4. Magnetic Resonance Cholangio-Pancreatography (MRCP) with Biopsy

Question 25

What is the purpose of the Liver Function Test (and Biochemistry)

Answer 25

1. To assess for Alkaline Phosphatase Levels | 2. To look at the Blood Lipid and IgM content

Question 26

What is the purpose of the Serum Anti-Mitochondrial Antibody test?

Answer 26

To look for the associated antibodies with this condition

Question 27

What is the purpose of the Abdominal Ultrasound / MRCP with Biopsy?

Answer 27

To assess if the Common Bile Duct is effected

Question 28

What is the treatment for Primary Biliary Cirrhosis?

Answer 28

1. Vitamin Supplements 2. Ursodeoxycholic Acid 3. Antihistamine / Cholestryamine

Question 29

What is the purpose of giving Vitamin Supplements?

Answer 29

As these can, sometimes, not be readily absorbed due to the decrease in Bile Secretion

Question 30

What Vitamins will need to be Supplemented?

Answer 30

1. Vitamin A 2. Vitamin D 3. Vitamin E 4. Vitamin K

Question 31

What is the purpose of giving Ursodeoxycholic Acid?

Answer 31

This improves Bilirubin and Aminotransferase Levels

Question 32

What is the purpose of giving Antihistamines / Cholestryamine?

Answer 32

Symptomatic Relief of Pruritus (itching)