Retrospective Study
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 28, 2020; 26(28): 4126-4139
Published online Jul 28, 2020. doi: 10.3748/wjg.v26.i28.4126
Primary sclerosing cholangitis associated colitis: Characterization of clinical, histologic features, and their associations with liver transplantation
John Aranake-Chrisinger, Themistocles Dassopoulos, Yan Yan, ILKe Nalbantoglu
John Aranake-Chrisinger, ILKe Nalbantoglu, Department of Pathology and Immunology, Lauren V. Ackerman Laboratory of Surgical Pathology, Washington University in St. Louis, School of Medicine, MO 63110, United States
Themistocles Dassopoulos, Baylor Center for IBD, Baylor University Medical Center, Dallas, TX 75246, United States
Yan Yan, Departments of Surgery and Biostatistics, Washington University in St. Louis, School of Medicine, MO 63110, United States
ILKe Nalbantoglu, Department of Anatomic Pathology, Yale University School of Medicine, New Haven, CT 06525, United States
Author contributions: Aranake-Chrisinger J, Nalbantoglu I, and Dassopoulos T came up with the study design; Aranake-Chrisinger J and Nalbantoglu I collected data and organized the manuscript; Yan Y performed the statistical analysis.
Institutional review board statement: This study was reviewed and approved by the Washington University in St. Louis, School of Medicine.
Informed consent statement: The subjects in this study were not required to give informed consent due to retrospective nature of the study and utilization of anonymous data utilization.
Conflict-of-interest statement: We have no financial relationships to disclose.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: ILKe Nalbantoglu, MD, Associate Professor, Physician, Department of Pathology, Yale University School of Medicine, 20 York Street, EP2-608B, New Haven, CT 06510, United States.
Received: March 30, 2020
Peer-review started: March 30, 2020
First decision: April 25, 2020
Revised: June 8, 2020
Accepted: July 15, 2020
Article in press: July 15, 2020
Published online: July 28, 2020
Research background

Primary Sclerosing cholangitis (PSC) associated inflammatory bowel disease (IBD) is a unique form of IBD seen in patients with PSC. It has been characterized as to have right sided predominance in colon with less severe active inflammation. Most cases of PSC-IBD are classified as ulcerative colitis (UC), whereas rare cases exhibit features of Crohn disease. There is thought to be a link between the IBD severity and the progression of PSC.

Research motivation

The need for better characterization of the pathologic findings in PSC-IBD patients and its association with liver transplantation may further support the “gut-liver axis” theory and has potential clinical implications. In addition, little is known regarding PSC-Crohn disease and its clinical outcomes.

Research objectives

The primary aims in this study were to characterize the colon and ileal findings in PSC patients at a tertiary care center, better define the histologic features of PSC-IBD, and explore if there is any correlation between the intestinal disease and liver transplant status, since this can impact patient management.

Research methods

This retrospective study was conducted in a single tertiary care center. Based on data search, cases with PSC and lower gastrointestinal biopsies were identified. Care was taken to examine the most inflamed biopsy. The hematoxylin and eosin slides were re-reviewed and several morphologic features were recorded. Pertinent clinical data was collected.

Research results

Our study confirmed the previously reported histologic findings in PSC-UC patients including a predominantly right sided involvement with overall less severity. We also described detailed histologic features of PSC-Crohn disease (CD) patients. None of the PSC-CD patients required liver transplantation, in contrast to ten PSC-UC patients. In our study, there was no correlation between the clinical parameters or treatment and orthotopic liver transplantation (OLT). When all PSC-IBD patients were analyzed together, severe left sided colitis correlated with the need for OLT.

Research conclusions

In our cohort, PSC-IBD patients with severe left sided and rectal disease required OLT more commonly than other PSC-IBD patients. This is rather interesting, since it may indicate that these patients are at increased risk for progression of their liver disease and this has not been reported before.

Research perspectives

The findings in this study further support the notion that gut and liver interact through several different mechanisms. Our results raise the possibility that an only a subset of patients with PSC-IBD (severe disease activity in left colon in our cohort) may be at increased risk for faster progression of liver disease, and eventually receive OLT. However, the contribution of other factors such as microbiome, genetic underpinnings, or others remain unanswered and should be further studied.