what is 1ry biliary cirrhosis
condition where immune system attacks small bile ducts within live r
what happens
obstruction of outflow of bile (cholestasis)
back pressure of bile obstruction ultimately leads to fibrosis, scarring and liver failure
presentation
fatigue pruritus GI disturbance jaundice pale stools xanthoma xanthelasma signs of cirrhosis
associations
middle aged women
other autoimmune conditions
rheumatoid conditins
diagnosis
LFTs
autoantibodies
liver biopsy
LFT findings
alkaline phosophhatase is first enzyme to be raised
other enzymes and bilirubin in later disease
autoantibodies
anti-mitochondrial antibodies
anti-nuclear antibodies
treatment
ursodeoxycholic acid - reduce cholesterol absioprtion
colestyramine
important end results of disease
advanced liver cirrhosis
portal hypertension