Primary Biliary Cholangitis Flashcards
What type of condition is primary biliary cholangitis?
Autoimmune condition
What does primary biliary cirrhosis cause?
Scarring and inflammation of the bile ducts, eventually leading to liver cirrhosis.
What organ is primarily affected in primary biliary cirrhosis?
The liver
Who is commonly affected by primary biliary cirrhosis?
Middle aged females
How might primary biliary cholangitis present in the early stages?
asymptomatic (e.g. raised ALP on routine LFTs)
Fatigue
Pruritus
What is a key clinical feature of primary biliary cholangitis?
hyperpigmentation over pressure points
xanthelasmas, xanthomata
What conditions is primary biliary cholangitis associated with?
Sjogren’s syndrome (up to 80% of patients)
rheumatoid arthritis
systemic sclerosis
thyroid disease
How can primary biliary cholangitis be diagnosed?
Immunology
Imaging
What would you find on immunology that would suggest primary biliary cholangitis?
anti-mitochondrial antibodies (AMA) M2 subtype
Smooth muscle antibodies in 30% of patients
raised serum IgM
What imaging can be done for suspected primary biliary cholangitis?
Right upper quadrant ultrasound or magnetic resonance cholangiopancreatography (MRCP)
What is the first line management of prmary biliary cholangitis?
Ursodeoxycholic acid
What is the function of ursodeoxycholic acid?
Slows down progression of the disease
What is used for the management of pruritis?
Cholestyramine
What are the possible complications of primary biliary cholagitis?
cirrhosis
osteomalacia and osteoporosis
Increased risk of hepatocellular carcinoma
