3.6. Liver + Biliary Tree Disease - Primary Biliary Cirrhosis Flashcards
What is the known Aetiology of Primary Biliary Cirrhosis?
Trick Question - It is Unknown
What is the Suspected Aetiology of Primary Biliary Cirrhosis?
An autoimmune disorder
Why is this thought to be an Autoimmune disorder?
Due to immunological mechanisms playing a part
Is Primary Biliary Cirrhosis more common in Males or Females?
Females
In what age group is Primary Biliary Cirrhosis most apparent?
40-50 years old
What is the term used for an early, preceding lesion?
Chronic, Non-Suppurative, Destructive Cholangitis
What does Non-Suppurative mean?
Without pus formation on inflammation
What does the preceding lesion highlight?
That it is only Late-Stage which ends in Cirrhosis
What is the Pathology of Primary Biliary Cirrhosis?
There is a progressive destruction of the Small Bile Ducts, eventually leading to Cirrhosis
What is found in most patients with Primary Biliary Cirrhosis?
Serum Anti-Mitochondrial Antibodies (AMA positive disease)
What causes the initiation of the Pathology of Primary Biliary Cirrhosis?
It is most likely that an Environmental Factor acts on a Genetically Predisposed Host - via Molecular Mimicry - initiating autoimmunity
What does the Molecular Mimicry (initiating the Autoimmunity) cause?
Granulomatous Formation
What does Granulomatous Formation result in?
Reduction in the effectiveness of the Intrahepatic Ducts, eventually leading to Death and Cirrhosis
What are the Clinical Signs of Primary Biliary Cirrhosis?
- Serum Anti-Mitochondrial Antibodies
- A Raised Serum Alkaline Phosphatase (ALP)
- Hepatomegaly
- Xanthelasma (and other Cholesterol Deposits)
Why is there raised Serum Alkaline Phosphatase (ALP)?
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
Why does Hepatomegaly occur?
Due to the damage and inflammation of the Liver
What is Xanthelasma?
A Yellowish Fat Deposition around the Skin of the Eye
Why does Xanthelasma occur (and other Cholesterol Deposits)?
Due to the Liver Inability to deal with Cholesterol any more
What are the Symptoms of Primary Biliary Cirrhosis?
- Asymptomatic (usually)
- Pruritus
- Jaundice
- Fatigue (and other generalized symptoms)
How is Primary Biliary Cirrhosis normally discovered, if Asymptomatic?
- On a routine examination for something else
2. Screening
What is Pruritus?
Itching
Why does Pruritus occur in Primary Biliary Cirrhosis?
Due to the Elevated Levels of Bilirubin in the Blood
What type of Jaundice will occur in Primary Biliary Cirrhosis?
Intrahepatic / Post-Hepatic Jaundice
What investigations are required for Primary Biliary Cirrhosis?
- Liver Function Test (and Biochemistry)
- Serum Anti-Mitochondrial Antibody (AMA) test
- Abdominal Ultrasound
- Magnetic Resonance Cholangio-Pancreatography (MRCP) with Biopsy
What is the purpose of the Liver Function Test (and Biochemistry)
- To assess for Alkaline Phosphatase Levels
2. To look at the Blood Lipid and IgM content
What is the purpose of the Serum Anti-Mitochondrial Antibody test?
To look for the associated antibodies with this condition
What is the purpose of the Abdominal Ultrasound / MRCP with Biopsy?
To assess if the Common Bile Duct is effected
What is the treatment for Primary Biliary Cirrhosis?
- Vitamin Supplements
- Ursodeoxycholic Acid
- Antihistamine / Cholestryamine
What is the purpose of giving Vitamin Supplements?
As these can, sometimes, not be readily absorbed due to the decrease in Bile Secretion
What Vitamins will need to be Supplemented?
- Vitamin A
- Vitamin D
- Vitamin E
- Vitamin K
What is the purpose of giving Ursodeoxycholic Acid?
This improves Bilirubin and Aminotransferase Levels
What is the purpose of giving Antihistamines / Cholestryamine?
Symptomatic Relief of Pruritus (itching)
