primary biliary colangitis Flashcards
who is primary billiard colangitis usually seen in?
middle aged females
aetiology
interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis.
associations
sjogren’s syndrome
rheumatoid arthiritis
systemic sclerosis
thyroid disease
clinical feautures
cholestatic jaundice
hyperpigmentation over pressure points
RUQ pain
clubbing
immunology
anti-microbial antibodies M2 subtypes are present in 98% of patients and are highly specific.
smooth muscle antibodies in 30% of patients.
raised serum IgM.
imaging
required before diagnosis to exclude an extra hepatic biliary obstruction
first-line management
ursodeoxycholic acid - slows disease progression and improves symptoms.
over treatment options
pruritus
fat-soluble vitamin
liver transplantation
complications
cirrhosis
portal hypertension
ascites
visceral haemorrhage