Primary Biliary Cholangitis Flashcards
What is primary biliary cholangitis?
autoimmune condition where the immune system attacks the small bile ducts in the liver, resulting in obstructive jaundice and liver disease.
What ducts are first affected in primary biliary cholangitis?
Intralobar ducts (canals of herring)
Where does inflammation occur in primary biliary cholangitis?
Small bile ducts
Epithelial cells
What 3 things are excreted through the bile ducts into the intestines?
Bile acid
Bilirubin
Cholesterol
What symptom does a rise in bile acids cause?
Pruritis (itching)
What symptom does a rise in bilirubin cause?
Jaundice
What clinical sign is seen with a rise in cholesterol?
Xanthelasma
What is the function of bile acids?
Digestion of fats
What happens when there is reduced or absent bile acids?
Malabsorption of fat
Greasy stools
What 2 symptoms are caused by a lack of bilirubin being excreted?
Pale stools
Dark urine
What is the typical patient with primary biliary cholangitis?
White woman aged 40-60 with another autoimmune/rheumataogical disease
What are the presenting features of primary biliary cholangitis? (6)
• Fatigue
• Pruritus (itching)
• Gastrointestinal symptoms and abdominal pain
• Jaundice
• Pale, greasy stools
• Dark urine
What can be seen on examination in primary biliary cholangitis? (4)
○ Xanthoma and xanthelasma (cholesterol deposits)
○ Excoriations (scratches on the skin due to itching)
○ Hepatomegaly
○ Signs of liver cirrhosis and portal hypertension in end-stage disease (e.g., splenomegaly and ascites)
What is seen on LFTs in primary biliary cholangitis?
Raised ALP
Bilirubin raised later in disease
What autoantibodies are found in primary biliary cholangitis? (2)
○ Anti-mitochondrial antibodies (AMA) are the most specific to PBC and form part of the diagnostic criteria
○ Anti-nuclear antibodies are present in about 35% of patients - not very specific
What immunoglobulin is raised in primary biliary cholangitis?
IgM
What investigation is used in diagnosing and staging primary biliary cholangitis?
Liver biopsy
What is the first line treatment in primary biliary cholangitis? How does it work?
Ursodeoxycholic acid
It is a non-toxic, hydrophilic bile acid that protects the cholangiocytes from inflammation and damage.
It makes the bile less harmful to the epithelial cells of the bile ducts.
Reduces intestinal absorption of cholesterol
It slows the disease progression and improves outcomes.
What drug is used as second line treatment of primary biliary cholangitis?
Obeticholic acid
What drug is used for symptoms of Pruritis?
Colestyramine
What are the complications of primary biliary cholangitis? (7)
Liver cirrhosis -> HCC
Fat-soluble vitamin deficiency (A, D, E and K)
Osteoporosis
Hyperlipidaemia (raised cholesterol)
Sjögren’s syndrome (dry eyes, dry mouth and vaginal dryness)
Connective tissue diseases (e.g., systemic sclerosis)
Thyroid disease
