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Changing nomenclature for PBC: from ‘cirrhosis’ to ‘cholangitis’
  1. Ulrich Beuers1,
  2. M Eric Gershwin2,
  3. Robert G Gish3,
  4. Pietro Invernizzi4,
  5. David E J Jones5,
  6. Keith Lindor6,
  7. Xiong Ma7,
  8. Ian R Mackay8,
  9. Albert Parés9,
  10. Atsushi Tanaka10,
  11. John M Vierling11,
  12. Raoul Poupon12
  1. 1Department of Gastroenterology & Hepatology and Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
  2. 2Division of Rheumatology, Allergy and Clinical Immunology, The University of California School of Medicine, Davis, California, USA
  3. 3Division of Gastroenterology & Hepatology, Department of Medicine, Stanford University, Stanford, California, USA
  4. 4Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano (Milan), Italy
  5. 5Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
  6. 6College of Health Solutions, Arizona State University, Phoenix, Arizona, USA
  7. 7Division of Gastroenterology & Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Institute of Digestive Disease, Shanghai, China
  8. 8Department of Biochemistry & Molecular Biology, Monash University, Clayton, Victoria, Australia
  9. 9Liver Unit, Hospital Clinic, IDIBAPS, CIBERehd, University of Barcelona, Barcelona, Spain
  10. 10Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
  11. 11Departments of Medicine and Surgery, Baylor College of Medicine, Houston, Texas, USA
  12. 12Service d'Hépatologie, Reference Center for Inflammatory Biliary Diseases, Saint-Antoine Hospital, Paris, France
  1. Correspondence to Ulrich Beuers, Department of Gastroenterology & Hepatology, Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, Meibergdreef 9, Amsterdam 1100 DE, The Netherlands; u.h.beuers{at}amc.uva.nl http://creativecommons.org/licenses/by/4.0/. This article is being published jointly in American Journal of Gastroenterology, Clinical Gastroenterology and Hepatology, Clinics and Research in Hepatology and Gastroenterology, Digestive & Liver disease, Gastroenterology, Gut, Hepatology and Journal of Hepatology.

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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 …

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