Primary biliary cirrhosis [Primaire biliaire cirrose.]

In the Netherlands there are probably several thousands of patients with primary biliary cirrhosis (PBC), a slowly progressive liver disease mainly affecting middle-aged women. PBC has characteristics of an autoimmune disease but its precise aetiology remains unknown. Fatigue and pruritus are the main symptoms but patients may also be asymptomatic. The diagnosis can be established through the presence of cholestatic liver test abnormalities, antimitochondrial antibodies and diagnostic or compatible findings upon liver biopsy. Currently most patients are diagnosed with early disease. When treated with ursodeoxycholic acid these patients have a normal prognosis.