• 6-MERCAPTOPURINE

The disease entity today widely called ‘primary biliary cirrhosis’ was first described by Addison and Gull in 18511 and Hanot in 1876.2 One hundred years after its first description, MacMahon and Thannhauser3 proposed the term ‘xanthomatous biliary cirrhosis’ for this disease based on the typical xanthoma formation with accumulation of cholesterol esters in the skin around the eyes in association with inflammatory destruction of small intrahepatic bile ductules leading to a biliary type cirrhosis. Xanthoma formation, however, is not a very common sign in this disorder. This may be the reason why the term ‘primary biliary cirrhosis’, proposed 1 year later for the same disorder by Ahrens et al,4 gained wider acceptance when most patients were presenting with advanced liver disease.

Dame Sheila Sherlock, already in 1959, opposed the term ‘primary biliary cirrhosis’ as many of her patients were free of cirrhosis at the time of diagnosis and the mean survival was 5 and a half years (3–11) even for the fatal cases, whereas many asymptomatic patients would survive more than 10 years.5 The term ‘primary biliary cirrhosis’ remained an issue of concern as reflected by the name change proposal of Rubin, Schaffner and Popper in 1965 with their paper ‘Primary biliary cirrhosis—Chronic non-suppurative destructive cholangitis’.6 Sherlock7 wisely commented on this new name: “…a better one, although it is unlikely that it will replace the more popular, although inaccurate, one of primary biliary cirrhosis”. She was right, again. And even 40 years later, the European8 and American9 Clinical Practice Guidelines still used the term ‘primary biliary cirrhosis’ even though it was an anachronism and did not accurately reflect the natural history of disease in the vast majority of patients as it is today.

The early diagnosis of primary biliary cirrhosis has …