‘Stat rosa pristina nomine, nomina nuda tenemus’ (“The rose exists well before its name; names are only a pale representation of the real nature of things”) ‘The Name of the Rose’ by Umberto Eco.Evolution of language is as old as language itself and the ever-changing terminology in the medical sciences remains an immediate challenge for all of us except perhaps the very newest trainees who will soon find their own common terms to have all too soon transitioned to anachronisms. Such evolution in language is indeed relevant to the diagnosis of non-alcoholic steatohepatitis-related cirrhosis (‘NASH cirrhosis’) and ‘cryptogenic cirrhosis’. The recognition that NASH cirrhosis progresses to an advanced state characterized by loss of a vital histological hallmark ‘steatosis’, and thus to loss of an even more foundational biopsy finding of ‘steatohepatitis’ was a conceptual sea-change.
Early epidemiological studies indicated that this process was characteristic of the transition of NASH cirrhosis to cryptogenic cirrhosis.
- Powell E.E.
- Cooksley W.G.
- Hanson R.
- Searll J.
- Halliday J.W.
- Powell L.W.
The natural history of nonalcoholic steatohepatitis: a follow-up study of forty-two patients for up to 21 years.
Hepatology. 1990; 11: 74-80
- Caldwell S.H.
- Oelsner D.H.
- Iezzoni J.C.
- Hespenheide E.E.
- Battle E.H.
- Driscoll C.J.
Cryptogenic cirrhosis: clinical characterization and risk factors for underlying disease.
Hepatology. 1999; 29: 664-669
- Poonawala A.
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Prevalence of obesity and diabetes in patients with cryptogenic cirrhosis: a case-control study.
Hepatology. 2000; 32: 689-692
4Indeed, the progression of NASH is now known to be associated with loss of fat (perhaps through changes in blood flow and insulin exposure) and diminished serum aminotransferases, albeit with increasing fibrosis.
- Clark J.M.
- Diehl A.M.
Nonalcoholic fatty liver disease: An under-recognized cause of cryptogenic cirrhosis.
JAMA. 2003; 289: 3000-3004
In the absence of a prior diagnosis of NASH or at least non-alcoholic fatty liver disease (NAFLD), liver histology at this stage appears as cirrhosis of unknown cause or ‘cryptogenic cirrhosis’.
- Caldwell S.H.
- Crespo D.M.
The spectrum expanded: cryptogenic cirrhosis and the natural history of nonalcoholic fatty liver disease.
J Hepatol. 2004; 40: 578-584
Following a transition period from around 2000 to 2014, the term ‘cryptogenic’ cirrhosis has come to be commonly equated to ‘NASH cirrhosis’. This may well be the most likely situation in the majority of cases, however, the authors of the present study raise a wise cautionary flag regarding the need to consider other possibilities.
- Caldwell S.H.
- Lee V.D.
- Kleiner D.E.
- Al-Osaimi A.M.
- Argo C.K.
- Northup P.G.
- et al.
NASH and cryptogenic cirrhosis: a histological analysis.
Ann Hepatol. 2009; 8: 346-352
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- The natural history of nonalcoholic steatohepatitis: a follow-up study of forty-two patients for up to 21 years.Hepatology. 1990; 11: 74-80
- Cryptogenic cirrhosis: clinical characterization and risk factors for underlying disease.Hepatology. 1999; 29: 664-669
- Prevalence of obesity and diabetes in patients with cryptogenic cirrhosis: a case-control study.Hepatology. 2000; 32: 689-692
- Nonalcoholic fatty liver disease: An under-recognized cause of cryptogenic cirrhosis.JAMA. 2003; 289: 3000-3004
- The spectrum expanded: cryptogenic cirrhosis and the natural history of nonalcoholic fatty liver disease.J Hepatol. 2004; 40: 578-584
- NASH and cryptogenic cirrhosis: a histological analysis.Ann Hepatol. 2009; 8: 346-352
Thuluvath PJ, Kantsevoy S, Thuluvath AJ, Savva Y. Is cryptogenic cirrhosis different from NASH cirrhosis? J Hepatol 2018;68:519–525.
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Published online: December 13, 2017
Accepted: December 5, 2017
Received: December 5, 2017See Article, pages 519–525
© 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.