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Editorial| Volume 68, ISSUE 3, P391-392, March 2018

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Cryptogenic vs. NASH-cirrhosis: The rose exists well before its name....

Published:December 13, 2017DOI:https://doi.org/10.1016/j.jhep.2017.12.006
      ‘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.
      • 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.
      Early epidemiological studies indicated that this process was characteristic of the transition of NASH cirrhosis to cryptogenic cirrhosis.
      • 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.
      • Poonawala A.
      • Nair S.P.
      • Thuluvath P.J.
      Prevalence of obesity and diabetes in patients with cryptogenic cirrhosis: a case-control study.
      • Clark J.M.
      • Diehl A.M.
      Nonalcoholic fatty liver disease: An under-recognized cause of cryptogenic cirrhosis.
      Indeed, 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.
      • Caldwell S.H.
      • Crespo D.M.
      The spectrum expanded: cryptogenic cirrhosis and the natural history of nonalcoholic fatty liver disease.
      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.
      • 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.
      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.

      Linked Article

      • Is cryptogenic cirrhosis different from NASH cirrhosis?
        Journal of HepatologyVol. 68Issue 3
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          Cryptogenic cirrhosis (CC) is a diagnosis of exclusion when there is no other known identifiable etiology.1 About two decades ago, when non-alcoholic steatohepatitis (NASH) was emerging as a common cause of chronic liver disease in the Western world, we and others had suggested that many patients with CC may have evolved from NASH.2–4 These observations were made based on patient characteristics and the higher prevalence of risk factors including type 2 diabetes mellitus (DM), obesity or metabolic syndrome in those with CC, since histology was often not helpful once a patient with NASH had progressed to cirrhosis.
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