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Th e disease entity today widely called ‘primary biliary cirrhosis’ was fi rst described by Addison & Gull in 1851 ( 1 ) and Hanot in 1876 ( 2 ). One hundred years aft er its fi rst description, MacMahon & Th annhauser 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 infl ammatory destruction of small intrahepatic bile ductules leading to a biliary type cirrhosis ( 3 ). Xanthoma formation, however, is not a very common sign in this disorder. Th is may be the reason why the term ‘primary biliary cirrhosis’, proposed one 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 ). Th e term ‘primary biliary cirrhosis’ remained an issue of concern as refl ected by the name change proposal of Rubin, Schaff ner and Popper in 1965 with their paper ‘Primary biliary cirrhosis—Chronic non-suppurative destructive cholangitis’ ( 6 ). S. Sherlock 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’’ ( 7 ). She was right, again. And even 40 years later, the European ( 8 ) and American ( 9 ) Clinical Practice Guidelines still used the term ‘primary biliary cirrhosis’ even though it was an anachronism and did not accurately refl ect the natural history of disease in the vast majority of patients as it is today. Th e early diagnosis of primary biliary cirrhosis has dramatically improved with the more accurate measurements of markers of cholestasis and improvements in the detection of the classic serologic hallmark, anti-mitochondrial antibodies. Furthermore, the prognosis has dramatically improved with the introduction of orthotopic liver transplantation in the 1970s and 1980s, and of ursodeoxycholic acid (UDCA, 13–15 mg/kg daily) treatment in the 1980s and 1990s. Today, two out of three patients diagnosed with primary biliary cirrhosis and treated with UDCA have an expected survival not diff erent from the general population and only a minority will ever develop cirrhosis. Th e pathogenesis of primary biliary cirrhosis remains enigmatic although enormous progress has been made in unravelling genetic, immunological and pathophysiological molecular mechanisms involved ( 10–13 ). Th is has also led to new therapeutic approaches which are now under evaluation ( 14,15 ). On 23–24 May, 2014, the 2nd European Association for the Study of the Liver (EASL) Monothematic Conference on Primary Biliary Cirrhosis took place in Milan, Italy. On this occasion, patient representatives from the UK and Germany, Robert Mitchell-Th ain and Ingo van Th iel, representing numerous national patient groups from diff erent parts of the world requested to change the name of ‘primary biliary cirrhosis’ to ‘‘correct the inaccuracy’’ and ‘‘remove the cirrhosis stigma’’ as well as all the misunderstanding, disadvantages and discriminations emanating from this misnomer in daily life of the patients.

Changing Nomenclature for PBC: From ‘Cirrhosis’ to ‘Cholangitis’