Changing Nomenclature for PBC: From 'Cirrhosis' to 'Cholangitis'

Ulrich H W Beuers, M Eric Gershwin, Robert G Gish, Pietro Luigi Invernizzi, David E J Jones, Keith D Lindor, Xiong Ma, Ian R Mackay, Albert Pares, Atsushi Tanaka, John M Vierling, Raoul Poupon

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55 Citations (Scopus)

Abstract

The disease entity today widely called `primary biliary cirrhosis was first described by Addison Gull in 18511 and Hanot in 1876. One hundred years after its first description, MacMahon Thannhauser 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 one year later for the same disorder by Ahrens et al., gained wider acceptance when most patients were presenting with advanced liver disease.
Original languageEnglish
Pages (from-to)1627 - 1629
Number of pages3
JournalGastroenterology
Volume149
Issue number6
DOIs
Publication statusPublished - 2015

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