Primary Biliary Cholangitis Flashcards
What is another term for primary biliary cholangitis?
Primary biliary cirrhosis
What is primary biliary cholangitis?
It is defined as a chronic condition in which the immune system targets the interlobular bile ducts within the liver
What is another term for the interlobular bile ducts?
Canals of Herring
What is the pathophysiological consequence of primary biliary cholangitis?
This leads to progressive obstruction of bile outflow, referred to as cholestasis
This back-pressure of bile obstruction leads to fibrosis, cirrhosis and liver failure
What are the four risk factors of primary biliary cholangitis?
Female Gender
Middle Aged
Rheumatoid Conditions
Autoimmune Conditions
What three rheumatoid conditions are associated with primary biliary cholangitis?
Sjorgen’s syndrome
Systemic sclerosis
Rheumatoid arthritis
What two autoimmune conditions are associated with primary biliary cholangitis?
Thyroid disease
Coeliac disease
What are the seven clinical features associated with primary biliary cholangitis?
Pruritis
Jaundice
Xanthoma
Xanthelasmas
Right Upper Quadrant Pain
Steatorrhoea
Hepatosplenomegaly
How is pruritus a clinical feature of primary biliary cholangitis?
The cholestasis results in an increased level of bile acids in the blood
How is jaundice a clinical feature of primary biliary cholangitis?
The cholestasis results in an increased level of bilirubin in the blood
What is xanthoma?
It is defined as the nodular deposition of cholesterol in the skin/tendons
What is xanthelasma?
It is defined as a subtype of xanthoma, in which there is the deposition of cholesterol in the skin - usually around the eyelids
How are xanthoma and xanthelasma clinical features of primary biliary cholangitis?
The cholestasis results in an increased level of cholesterol in the blood
How is steatorrhoea a clinical features of primary biliary cholangitis?
The cholestasis results in a lack of bile acids in the gastrointestinal tract, which aid the digestion of fats
What three investigations are used to diagnose primary biliary cholangitis?
Blood Tests
Ultrasound Scan
Liver Biopsy
What six blood test results indicate primary biliary cholangitis?
Increased ALP Levels
Increased Bilirubin Levels
Increased Serum IgM Levels
Increased ESR Levels
Anti-Mitochondrial Antibodies (AMA) M2 Subtype
Smooth Muscle Antibodies (SMA)
Which antibody is most commonly associated with primary biliary cholangitis?
Anti-Mitochondrial Antibodies (AMA) M2 Subtype
How is an ultrasound scan used to investigate primary biliary cholangitis?
It is used to exclude an extrahepatic biliary obstruction related cause
How is a liver biopsy used to investigate primary biliary cholangitis?
It is used to confirm a diagnosis of primary biliary cholangitis and to stage the disease
What are the three pharmacological management options of primary biliary cholangitis?
Ursodeoxycholic Acid
Cholestyramine
Fat Soluble Vitamin Supplementation
What is the first line pharmacological management option of primary biliary cholangitis?
Ursodeoxycholic Acid
What is the mechanism of action of ursodeoxycholic acid?
It reduces the intestinal absorption of cholesterol
How is ursodeoxycholic acid used to manage primary biliary cholangitis?
It slows disease progression and provides symptomatic relief
What is the mechanism of action of cholestyramine?
It is a bile acid sequestrate, which binds to bile acids to prevent absorption in the gut
It therefore increases bile acid levels in the gut
How is cholestyramine used to manage primary biliary cholangitis?
It relieves pruritis features
What surgical management option is used in primary biliary cholangitis?
Liver transplant
When are liver transplants used to manage primary biliary cholangitis?
When the complication development of end stage liver disease occurs
OR
When bilirubin levels > 100
What are the seven complications of primary biliary cholangitis?
Liver Cirrhosis
Portal Hypertension
Hepatocellular Carcinoma
Osteoporosis
Osteomalacia
Distal Renal Tubular Acidosis
Hypothyroidism
What rule can be used to remember the key features of primary biliary cholangitis?
M rule
igM
anti-Mitochondria antibodies, M2 subtype
Middle aged females
