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Secondary sclerosing cholangitis after intensive care unit treatment: clues to the histopathological differential diagnosis

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Abstract

Secondary sclerosing cholangitis (SSC) is a chronic cholestatic disorder caused by mechanical, infectious, toxic, or ischemic factors. A new variant of SSC occurring after long-term treatment in intensive care units (ICU) has been recently described and characterized from the clinical point of view. The aim of this study was the histomorphological characterization of ICU-treatment-related SSC (ICU-SSC) and the definition of histological changes occurring over time based on the morphological findings. Liver biopsies of ten patients affected by ICU-SSC obtained at different time points (1.5 to 57 months) after the initial injury were analyzed. The main morphological alterations included degenerative changes of portal bile ducts, portal edema, inflammation, and fibrosis as well as biliary interface activity and bilirubinostasis. Perivenular necroses and bile infarcts were found in eight and six patients, respectively. Bile duct loss was not observed. No correlation between morphological features of biopsies and liver chemistry tests or outcome could be established. Based on the morphological observation, a possible disease-progression model starting with an initial damage of portal bile ducts (primary insult) with associated portal/periportal changes (inflammation, ductular reaction) and resulting in secondary parenchymal changes is proposed.

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References

  1. Maggs JR, Chapman RW (2007) Sclerosing cholangitis. Curr Opin Gastroenterol 23:310–316

    Article  PubMed  Google Scholar 

  2. Abdalian R, Heathcote EJ (2006) Sclerosing cholangitis: a focus on secondary causes. Hepatology 44:1063–1074

    Article  PubMed  Google Scholar 

  3. Bjornsson E, Chari ST, Smyrk TC et al (2007) Immunoglobulin G4 associated cholangitis: description of an emerging clinical entity based on review of the literature. Hepatology 45:1547–1554

    Article  PubMed  Google Scholar 

  4. Scheppach W, Druge G, Wittenberg G et al (2001) Sclerosing cholangitis and liver cirrhosis after extrabiliary infections: report on three cases. Crit Care Med 29:438–441

    Article  PubMed  CAS  Google Scholar 

  5. Engler S, Elsing C, Flechtenmacher C et al (2003) Progressive sclerosing cholangitis after septic shock: a new variant of vanishing bile duct disorders. Gut 52:688–693

    Article  PubMed  CAS  Google Scholar 

  6. Benninger J, Grobholz R, Oeztuerk Y et al (2005) Sclerosing cholangitis following severe trauma: description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms. World J Gastroenterol 11:4199–4205

    PubMed  Google Scholar 

  7. Jaeger C, Mayer G, Henrich R et al (2006) Secondary sclerosing cholangitis after long-term treatment in an intensive care unit: clinical presentation, endoscopic findings, treatment, and follow-up. Endoscopy 38:730–734

    Article  PubMed  CAS  Google Scholar 

  8. Gelbmann CM, Rummele P, Wimmer M et al (2007) Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients. Am J Gastroenterol 102:1221–1229

    Article  PubMed  Google Scholar 

  9. Kulaksiz H, Heuberger D, Engler S et al (2008) Poor outcome in progressive sclerosing cholangitis after septic shock. Endoscopy 40:214–218

    Article  PubMed  CAS  Google Scholar 

  10. Van Eyken P, Sciot R, Desmet VJ (1989) A cytokeratin immunohistochemical study of cholestatic liver disease: evidence that hepatocytes can express ‘bile duct-type’ cytokeratins. Histopathology 15:125–135

    PubMed  Google Scholar 

  11. Green RM, Flamm S (2002) AGA technical review on the evaluation of liver chemistry tests. Gastroenterology 123:1367–1384

    Article  PubMed  Google Scholar 

  12. Geier A, Fickert P, Trauner M (2006) Mechanisms of disease: mechanisms and clinical implications of cholestasis in sepsis. Nat Clin Pract Gastroenterol Hepatol 3:574–585

    Article  PubMed  CAS  Google Scholar 

  13. Lefkowitch JH (1982) Bile ductular cholestasis: an ominous histopathologic sign related to sepsis and "cholangitis lenta". Hum Pathol 13:19–24

    Article  PubMed  CAS  Google Scholar 

  14. Umemura T, Zen Y, Hamano H et al (2007) Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology 46:463–471

    Article  PubMed  CAS  Google Scholar 

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Conflict of interest statement

We declare that we have no conflict of interest.

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Authors and Affiliations

  1. Institute of Pathology, Helmholtz Zentrum München-German Research Center for Environmental Health, Oberschleissheim, Germany

    Irene Esposito

  2. Institute of Pathology, Technische Universität München, Munich, Germany

    Irene Esposito

  3. Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220, 69120, Heidelberg, Germany

    Irene Esposito, Andrea Kubisova & Peter Schirmacher

  4. Department of Internal Medicine, Division of Gastroenterology, University of Heidelberg, Heidelberg, Germany

    Adolf Stiehl

  5. Department of Internal Medicine, University of Ulm, Ulm, Germany

    Hasan Kulaksiz

Authors
  1. Irene Esposito
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  2. Andrea Kubisova
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  3. Adolf Stiehl
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  4. Hasan Kulaksiz
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  5. Peter Schirmacher
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Correspondence to Peter Schirmacher.

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Esposito, I., Kubisova, A., Stiehl, A. et al. Secondary sclerosing cholangitis after intensive care unit treatment: clues to the histopathological differential diagnosis. Virchows Arch 453, 339–345 (2008). https://doi.org/10.1007/s00428-008-0654-1

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  • DOI: https://doi.org/10.1007/s00428-008-0654-1

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