Flashcards in Primary Biliary Cirrhosis Deck (14)
anti-mitochondrial antibodies (AMAs)
unknown environmental triggers + genetic predisposition --> loss of immune tolerance to self-mitochonidal proteins
interlobular bile ducts damaged by chronic autoimmune granulomatous inflammation
may --> fibrosis, cirrhosis and portal HTN
Portal vein, hepatic artery, but no bile duct.
Immune damage directed to the small bile ducts
90% are women!
often incidental finding of raised alk phos on routine LFT
lethargy, sleepiness and pruritus may precede jaundice by years
jaundice, skin pigmentation, hepatosplenomegaly
malab of fat-soluble vitamins due to cholestasis and reduced billirubin in gut lumen --> osteomalacia and coagulopathy
raised alk phos, yGT
mildly raised AST & ALT
raised serum cholesterol
what does ultrasound exclude?
extra hepatic cholestasis
Tx of symptoms
itching - colestyramine
diarrhoea - codeine phosphate
osteoporosis prevention - bisphosphonates
fatigue - modafinil, vitsADEK supplements
what is the only routine Tx?
improves liver enzymes, bilirubin
what improves biochemical and histological disease but has side effects?