Primary biliary cirrhosis

Primary biliary cirrhosis is an autoimmune cholestatic liver disease which is diagnosed when 2 of the following 3 criteria are met: 

• At least 6 months biochemical evidence of cholestatic liver disease 
• Anti-mitochondrial antibody (AMA) positive 
• Characteristic histological features on liver biopsy 
• destruction of small and medium sized bile ducts
• non-supprative cholangitis

Females > males, 10:1 Onset usually around 5th decade of life

Around 2/3 of cases of primary biliary cirrhosis are diagnosed on the basis of blood tests, with the patient being otherwise asymptomatic.  Other presentations are of pruritis (classically worse at night) and fatigue.

Blood tests 

• LFTs show 
• Raised ALP and gammaGT 
• ALT, AST and bilirubin may be raised
• Raised PT only in advanced disease 
• AMA – 90% sensitive, 95% specific
• ANA – non-specific, present in 30%

Complications 

• Cirrhosis
• If cirrhosis present increased risk of hepatocellular carcinoma 
• Portal hypertension 
• Osteoporosis (1/3) 

Treatment

• ursodeoxycholic acid 
• liver transplant
• osteoporosis prophylaxis 

References

European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Management of cholestatic liver disease. Journal of Hepatology. 2009; 51: 237-267. 

Jones, D. Pathogenesis of primary biliary cirrhosis. Gut. 2007; 56(11): 1615-1624. 

Nguyen, D., Juran, B. and Lazaridis, K. Primary biliary cirrhosis. Best Pract Res Clin Gastroenterol. 2010; 24(5): 647-654.