Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

Lessons from a patient with an unusual hepatic overlap syndrome

Nature Clinical Practice Gastroenterology & Hepatology volume 4, pages 635–640 (2007)Cite this article

Abstract

Background A 24-year-old white male was diagnosed as having Crohn's disease by clinical, endoscopic and histological assessments, and long-term remission was successfully induced with short-course prednisolone. While the Crohn's disease was still in remission, the patient presented with cholestasis and hyperbilirubinemia but normal alanine aminotransferase levels, negative results for serological tests for infectious causes of hepatopathy and normal γ-globulin levels.

Investigations Repeated laboratory and serological tests, ultrasonography, endoscopic retrograde cholangiopancreatographies, CT scan, magnetic resonance cholangiopancreatography and liver biopsies.

Diagnosis Autoimmune hepatitis–primary sclerosing cholangitis (AIH–PSC) overlap syndrome on the background of PSC with a rare course of sequential manifestations of the different disease components.

Management Combined immunosuppression with prednisolone and treatment with ursodeoxycholic acid (UDCA), endoscopic treatment of dominant stenoses, bile duct stent implantation, and close follow-up.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Histological and cholangiographic features.
Figure 2: Graph showing the course of serum liver tests.
Figure 3: Diagnostic pathways in autoimmune liver disease.

References

  1. Beuers U and Rust C (2005) Overlap syndromes. Semin Liver Dis 25: 311–320

    Article  Google Scholar 

  2. Van Buuren HR et al. (2000) High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis. J Hepatol 33: 543–548

    Article  CAS  Google Scholar 

  3. Kaya M et al. (2000) Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system. J Hepatol 33: 537–542

    Article  CAS  Google Scholar 

  4. Abdo AA et al. (2002) Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology 36: 1393–1399

    Article  Google Scholar 

  5. Hong-Curtis J et al. (2004) Rapid progression of autoimmune hepatitis in the background of primary sclerosing cholangitis. J Clin Gastroenterol 38: 906–909

    Article  Google Scholar 

  6. Domschke WR et al. (2000) Sequential occurrence of primary sclerosing cholangitis and autoimmune hepatitis type III in a patient with ulcerative colitis: a follow up study over 14 years. Liver 20: 340–345

    Article  CAS  Google Scholar 

  7. Gregorio GV et al. (2001) Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 33: 544–553

    Article  CAS  Google Scholar 

  8. Boberg KM et al. (1996) Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis. Hepatology 23: 1369–1376

    Article  CAS  Google Scholar 

  9. Chapman RW (1991) Role of immune factors in the pathogenesis of primary sclerosing cholangitis. Semin Liver Dis 11: 1–4

    Article  CAS  Google Scholar 

  10. Czaja AJ et al. (2001) Shared genetic risk factors in autoimmune liver disease. Dig Dis Sci 46: 140–147

    Article  CAS  Google Scholar 

  11. Floreani A et al. (2005) Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol 100: 1516–1522

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. R Winograd and A Geier are Registrars in the Department of Pathology, LE Sander is a Resident, A Koch, at Aachen University Hospital, Aachen, Germany.,

    Leif E Sander, Alexander Koch, Ron Winograd & Andreas Geier

  2. C Trautwein is the Director in the Department of Internal Medicine III, at Aachen University Hospital, Aachen, Germany.,

    Axel Wellmann & Christian Trautwein

  3. A Donner and A Wellmann are Registrars in the Department of Pathology, at Aachen University Hospital, Aachen, Germany.,

    Andreas Donner

  4. C Gartung is the Director at Minden Hospital, Minden, Germany.,

    Carsten Gartung

Authors
  1. Leif E Sander
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Alexander Koch
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Carsten Gartung
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Ron Winograd
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Andreas Donner
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Axel Wellmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Christian Trautwein
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Andreas Geier
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Andreas Geier.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sander, L., Koch, A., Gartung, C. et al. Lessons from a patient with an unusual hepatic overlap syndrome. Nat Rev Gastroenterol Hepatol 4, 635–640 (2007). https://doi.org/10.1038/ncpgasthep0954

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpgasthep0954

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing