PSC associated inflammatory bowel disease: a distinct entity

ABSTRACT

Introduction

Primary sclerosing cholangitis (PSC) is a rare, chronic, and progressive cholestatic disease involving intra- and/or extrahepatic bile ducts. PSC in many patients results in end-stage liver diseases. Nearly 60% of the PSC patients suffer from concomitant inflammatory bowel diseases (IBDs). Classically, IBDs are divided into two principle types: Crohn’s disease (CD) and ulcerative colitis (UC). However, with growing knowledge, PSC-associated IBD (PSC-IBD) seems to be a rather distinct entity with specific genetics, clinical, and microbiota characteristics.

Areas covered

In this article, we aim to review the unique characteristics of PSC-IBD from clinical, genetic, and microbiota point of view.

Expert opinion

PSC-IBD’s unique characteristics contribute to the notion that it could be a distinct entity. Acknowledgment of PSC-IBD as a novel entity necessitates designing new clinical guidelines for diagnosis and developing novel therapies.

KEYWORDS:

• Primary sclerosing cholangitis
• PSC associated IBD
• inflammatory bowel diseases
• PSC-IBD

Article highlights

• PSC-IBD genetic susceptibility shows a distinct pattern compared to the IBD patients.

• PSC-IBD colonic involvement in contrast to IBD is right-sided and has a more severe course.

• Malignancy risk in PSC-IBD patients is higher compared to the IBD patients.

• Microbiota studies in PSC-IBD patients revealed a distinct microbiota pattern compared to the IBD patients.

Acknowledgments

The authors would like to thank all the staff of the Research Institute for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, and Regenerative Medicine Department, Rpyan Institute for their support.

Declaration of interests

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers in this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

Funding This paper was not funded.