Primary biliary cholangitis Flashcards
What is primary biliary cholangitis?
A chronic liver disorder typically seen in middle-aged females, previously referred to as primary biliary cirrhosis.
What is the female to male ratio in primary biliary cholangitis?
The female to male ratio is 9:1.
What is the suspected aetiology of primary biliary cholangitis?
It is thought to be an autoimmune condition.
What happens to the interlobular bile ducts in primary biliary cholangitis?
They become damaged by a chronic inflammatory process causing progressive cholestasis.
What is the classic presentation of primary biliary cholangitis?
Itching in a middle-aged woman.
What autoimmune conditions are associated with primary biliary cholangitis?
Sjogren’s syndrome, rheumatoid arthritis, systemic sclerosis, and thyroid disease.
What are early clinical features of primary biliary cholangitis?
May be asymptomatic, fatigue, pruritus, raised ALP on routine LFTs.
What are late clinical features of primary biliary cholangitis?
May progress to liver failure.
What are some clinical features of primary biliary cholangitis?
Cholestatic jaundice, hyperpigmentation, right upper quadrant pain, xanthelasmas, xanthomata, clubbing, hepatosplenomegaly.
What is the diagnostic test for primary biliary cholangitis?
Anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients.
What imaging is required before diagnosing primary biliary cholangitis?
Typically a right upper quadrant ultrasound or magnetic resonance cholangiopancreatography (MRCP) to exclude extrahepatic biliary obstruction.
What is the first-line management for primary biliary cholangitis?
Ursodeoxycholic acid.
What is used to manage pruritus in primary biliary cholangitis?
Cholestyramine.
What is a major indication for liver transplantation in primary biliary cholangitis?
If bilirubin > 100.
What are some complications of primary biliary cholangitis?
Cirrhosis, portal hypertension, ascites, variceal hemorrhage, osteomalacia, osteoporosis, and significantly increased risk of hepatocellular carcinoma.
