Primary Biliary Cirrhosis Flashcards
What is primary biliary cirrhosis?
An autoimmune condition where the immune system attacks the small bile ducts.
First part affected?
Intralobular ducts (Canals of Hering)
Consequences?
(1) Bile duct obstruction (cholestasis)
(2) Fibrosis, cirrhosis and liver failure
Chemicals that increase in the blood as a result of bile obstruction? Signs?
(1) Bile acids –> itching
(2) Bilirubin –> jaundice
(3) Cholesterol –> xanthomas
Signs of high bile acid?
- itching
- greasy stools (distrupts GI)
Signs of high bilirubin ?
- jaundice
- pale stools
Presentation?
(1) Extreme fatigue
(2) pruritis
(3) GI disturbance, abdo pain
(3) stools - greasy, pale
(4) xanthoma and xanthelasma
(5) Signs of liver disease
% of patients that are women and <40yrs?
25%
% of PBC that are men?
10%
Associations?
(1) middle aged women
(2) other autoimmune disorders (thyroid, coeliac)
(3) Rheumatoid conditions (RA, systemic sclerosis, Sjogrens)
PBC patients are at risk of what?
-HCC
Diagnosis is based on what investigations?
(1) LFTs - derranged
(2) Positive anti-mitochondrial antibodies (90%)
(3) Abdo US
(4) Liver biopsy - inflammation & scarring
Which liver enzyme is the first to rise?
Alkaline phosphatase (ALP)
Which other antibody may be present (35%)?
Anti-nuclear antibody
Other bloods that may be raised?
- ESR
- IgM
