Primary Biliary Cirrhosis Flashcards
What is Primary Biliary Cirrhosis?
Autoimmune attack on the small bile ducts
Which ducts are first to be affected?
Canals of Hering
What does PBC cause?
Cholestasis (bile outflow obstruction)
What does cholestasis lead to?
- Fibrosis
- Cirrhosis
- Liver failure
What does an increase in bile acids cause?
Pruritis
What does an increase in bilirubin cause?
Jaundice
What does an increase in cholesterol cause?
- Cardiovascular disease
- Xanthelasma (larger deposits are called xanthoma)
What does a lack of bile acids in the GI lumen cause?
Staetorrhoea
What does a lack of bilirubin in the stools cause?
Pale stools
How does PBC present?
- Fatigue
- Pruritis
- Jaundice
- GI Disturbance
- Xanthoma/xanthelasma
- Signs of Cirrhosis and Liver failure
What is PBC often associated with?
- Rheumatism e.g. RA, Sjogrens, SS)
- Other AI conditions
How is PBC diagnosed?
- LFTs
- Autoantibodies
- Immunoglobulins
- Liver biopsy
What may LFTs show in PBC?
- Raise ALP (obstructive pattern jaundice)
- Other liver enzymes, and bilirubin are raised later on in the disease course
Which autoantibodies are most specific to PBC, thus coming in the diagnostic criteria?
AMA (Anti-mitochondrial)
Which autoantibodies are present in around 35% of patients in PBC?
ANA (Anti-nuclear)
