Write about Primary Biliary Cirrhosis here.

• Interlobular bile ducts damaged by chronic granulomatous inflammation causing progressive cholestasis, cirrhosis and portal hypertension

• Autoimmune?

• F:M = 9:1

• Prevalence 4:100,000

• Peak presentation = 50 yrs

Tests:

• Raised Alk Phos, Raised gamma-GT, Mildly Raised AST and ALT

• Late disease raised bilirubin, reduced albumin, raised PT

• 98% are antimitochondrial antibody (AMA) M2 subtype +ve (very specific)

• Radiology: USS and ERCP to exclude extrahepatic cholestasis

• Liver biopsy: Granulomas around bile ducts -> cirrhosis

Management:

• Symptomatic:

• Pruritus - colestyramine

• Diarrhoea - codeine phosphate

• Osteoporosis prevention

• Specific:

• Fat-soluble vitamin prophylaxis 

• Consider ursodeoxycholic acid (UDCA) - (may slow disease progression and obviate need for transplant)

• Liver transplant - last recourse for patients with end-stage disease or intractable prurius

• Recurrence in graft may occur but does not affect graft success

Prognosis:

• Once jaundice develops survival < 2 yrs