Gastro - Primary Biliary Cholangitis Flashcards
What is primary biliary cholangitis?
Autoimmune condition where the intrahepatic ducts are attacked by immune system
Results in obstructive jaundice and liver disease
What is the pathophysiology of primary biliary cholangitis?
Intrahepatic ducts are attacked
Inflammation and damage to cholangiocytes
Over time this causes an obstruction of bile flow through these ducts
Reduced flow of bile (cholestasis)
Back pressure leads to liver fibrosis, cirrhosis and failure
How are bile acids, bilirubin and cholesterol excreted and what does obstruction cause?
Through bile ducts into the intestines
Build-up in blood leading to itching, jaundice and xanthelasma
What does raised cholesterol lead to?
Xanthelasma
Xanthomas (large nodular deposits of cholesterol in skin or tendons)
Increased risk of atherosclerosis and CVD
What do bile acids do?
Help fat digestion and absorption
Reduced bile acids cause abdominal pain, malabsorption of fat and greasy stools
Why do you get dark urine in primary biliary cholangitis?
Obstruction in intrahepatic ducts causing reduced excretion of bilirubin
Bilirubin excreted in urine causing dark urine
What is the typical patient with primary biliary cholangitis?
White woman
40-60 years old
Asymptomatic at diagnosis
Problem picked up on abnormal LFTs
How can primary biliary cholangitis present?
Fatigue
Pruritis
GI symptoms
Abdominal pain
Jaundice
Pale, greasy stools
Dark urine
What examination findings are present with primary biliary cholangitis?
Xanthoma and xanthelasma
Excoriations
Hepatomegaly
What investigations are used for primary biliary cholangitis?
LFTs
- Raised ALP (most notable with obstructive pathology)
Autoantibodies
- AMA most specific
- ANA (35% of the time)
Raised IgM
Ultrasound
- Rule out other pathology
Liver biopsy
- Diagnosing and staging
How is primary biliary cholangitis treated?
Ursodeoxycholic acid
Non-toxic
Hydrophilic bile acid
Protects cholangiocytes from inflammation and damage
Makes bile less harmful to epithelial cells of bile ducts, slows disease progression and improves outcomes
Asides from ursodeoxycholic acid, what other treatments are used for primary biliary cholangitis?
Obeticholic acid
Cholestyramine- for symptoms of pruritis, reduces intestinal absorption of bile acids
Replacement of fat-soluble vitamins
Immunosuppression
Liver transplant- end-stage liver disease
How does primary biliary cholangitis progress?
Varies significantly
Can live decades without symptoms
Most crucial complication is liver cirrhosis
What are some possible complications of primary biliary cholangitis?
Liver cirrhosis
Fat-soluble vitamin deficiency
Osteoporosis
Hyperlipidaemia
Sjogren’s syndrome
Connective tissue diseases
Thyroid disease
