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The IBD and PSC Phenotypes of PSC-IBD

  • Amanda Ricciuto
  • Binita M. Kamath
  • Anne M. Griffiths
Pediatric Gastroenterology (S Orenstein, Section Editor)
  • 320 Downloads
Part of the following topical collections:
  1. Topical Collection on Pediatric Gastroenterology

Abstract

Purpose of review

To review the characteristics of IBD and PSC that occur in association, as well as their reciprocal influences on disease evolution, in adult and pediatric populations.

Recent findings

IBD co-existing with PSC is genetically and clinically distinct from IBD alone. It is frequently characterized by pancolitis, rectal sparing, and possibly backwash ileitis, as well as a threefold increased risk of colorectal dysplasia. Adults and children with colitis and PSC appear to be at increased risk of active endoscopic and histologic disease in the absence of symptoms compared to individuals without PSC. PSC occurring with Crohn’s disease has been observed to be less severe than PSC co-existing with ulcerative colitis, independent of its association with small duct disease. Recent studies suggest that colectomy is associated with a decreased risk of recurrent PSC after liver transplantation, challenging the traditional teaching that PSC and IBD evolve independently.

Summary

While much about the gut-liver axis in PSC-IBD remains poorly understood, the IBD associated with PSC has a unique phenotype, of which subclinical inflammation is an important component. Additional research is needed to characterize further the potentially protective role of colectomy against recurrent PSC post-liver transplantation and to investigate the influence of IBD control and/or colectomy on PSC progression.

Keywords

Colectomy Inflammatory bowel disease (IBD) Liver transplantation Phenotype Primary sclerosing cholangitis (PSC) PSC-IBD 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Amanda Ricciuto
    • 1
  • Binita M. Kamath
    • 1
  • Anne M. Griffiths
    • 1
  1. 1.Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

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