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Curriculum based clinical reviews
Primary sclerosing cholangitis and the management of uncertainty and complexity
  1. Katherine Arndtz1,2,
  2. Gideon M Hirschfield1,2
  1. 1Centre for Liver Research,NIHR Birmingham Biomedical Research Centre, University of Birmingham, Birmingham, UK
  2. 2Centre for Rare Diseases, Institute of Translational Medicine, Birmingham Health Partners, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  1. Correspondence to Prof Gideon M Hirschfield, Centre for Liver Research, NIHR Birmingham Biomedical Research Centre, Institute of Immunology and Immunotherapy, University of Birmingham, Wolfson Drive B15 2TT, UK; g.hirschfield{at}bham.ac.uk

Abstract

Primary sclerosing cholangitis (PSC) is a rare chronic autoimmune disease with no effective therapy and a large unmet need for new treatments. Patients require significant healthcare resources over their lifetime with high rates of hospital admission, malignancy, liver transplantation and death. As a rare disease, expertise in management can be limited to large referral liver transplant programmes, and even then there is frequently variation in practice. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing patients with PSC and address related competencies in the 2010 Gastroenterology Curriculum.

  • autoimmune liver disease
  • cholestatic liver diseases
  • inflammatory bowel diseases
  • orthotopic liver transplantation
  • primarysclerosing colangitis

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Footnotes

  • Contributors GMH developed the idea for the article. KA wrote the article with supervision from GMH.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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