Topic Highlight
Copyright ©2014 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Mar 28, 2014; 20(12): 3245-3254
Published online Mar 28, 2014. doi: 10.3748/wjg.v20.i12.3245
Inflammatory bowel disease of primary sclerosing cholangitis: A distinct entity?
Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Hitoshi Sano, Katsuyuki Miyabe, Shuya Shimizu, Takashi Joh
Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Katsuyuki Miyabe, Shuya Shimizu, Takashi Joh, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya 467-8601, Japan
Hitoshi Sano, Department of Gastroenterology, Gifu Prefectual Tajimi Hospital, Gifu 507-0042, Japan
Author contributions: Nakazawa T, Naitoh I and Sano H designed the research; Hayashi K, Miyabe K and Shimizu S collected clinical data; Nakazawa T and Joh T analyzed data; Nakazawa T and Naitoh I wrote the paper.
Correspondence to: Takahiro Nakazawa, MD, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan. tnakazaw@med.nagoya-cu.ac.jp
Telephone: +81-52-8538211 Fax: +81-52-8520952
Received: August 28, 2013
Revised: January 8, 2014
Accepted: January 19, 2014
Published online: March 28, 2014
Processing time: 211 Days and 1.6 Hours
Abstract

This is a review of the characteristic findings of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) and their usefulness in the diagnosis of sclerosing cholangitis. PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD. IBD-associated with PSC (PSC-IBD) shows an increased incidence of pancolitis, mild symptoms, and colorectal malignancy. Although an increased incidence of pancolitis is a characteristic finding, some cases are endoscopically diagnosed as right-sided ulcerative colitis. Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon. The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition. The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are similar to those of PSC. The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.

Keywords: Primary sclerosing cholangitis; Primary sclerosing cholangitis; Inflammatory bowel disease; Inflammatory bowel disease-associated with primary sclerosing cholangitis; Immunoglobulin G4-related sclerosing cholangitis

Core tip: Inflammatory bowel disease (IBD)-associated with primary sclerosing cholangitis (PSC) (PSC-IBD) shows an increased incidence of pancolitis, mild symptoms, and colorectal malignancy. Although an increased incidence of pancolitis is a characteristic finding, some cases are endoscopically diagnosed as right-sided ulcerative colitis. Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon. The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are similar to those of PSC. The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.