Primary Biliary Cirrhosis Flashcards
In questions, what is the classic presentation for primary biliary cirrhosis?
Itching middle aged females
What is the pathophysiology of primary biliary cirrhosis?
Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis, which may eventually progress to cirrhosis.
What are some clinic features of primary biliary cirrhosis?
-
Early:
- May be asymptomatic (e.g. raised ALP on routine LFTs)
- Fatigue,
- Pruritus
- Cholestatic jaundice
- Hyperpigmentation, especially over pressure points
- Xanthelasmas, xanthomata
- Also: clubbing, hepatosplenomegaly
- Late: may progress to liver failure
Describe the M Rule for Primary Biliary Cirrhosis
- Raised IgM
- Anti-Mitochondrial antibodies M2 subtype are present in 98% of patients.
- Middle aged females
What are some complications of primary biliary cirrhosis?
- Malabsorption: osteomalacia, coagulopathy
- Sicca syndrome occurs in 70% of cases
- Portal hypertension: ascites, variceal haemorrhage
- Hepatocellular cancer (20-fold increased risk)
What other autoimmune disorders is primary biliary cirrhosis associated with?
- Sjogren’s syndrome (seen in up to 80% of patients)
- Rheumatoid arthritis
- Systemic sclerosis
- Thyroid disease
In questions, they may have a middle aged lady with raised ALP and a dry mouth (Xerostomia) and dry eyes. What is this?
PBC with associated Sicca syndrome
Although people with PBC can have Sjorgen’s syndrome, some can just have dry membranes. The term “sicca” refers to the dryness of the eyes and mouth.
What is the management pathway of PBC?
- Pruritus: cholestyramine (Binds to bile in the small intestine to prevent reabsorption and itching)
- Fat-soluble vitamin supplementation
- Ursodeoxycholic acid (Bile salts)
- Liver transplantation e.g. if bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem
