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Review
. 2014 Mar 28;20(12):3245-54.
doi: 10.3748/wjg.v20.i12.3245.

Inflammatory bowel disease of primary sclerosing cholangitis: a distinct entity?

Takahiro Nakazawa  1 , Itaru Naitoh  1 , Kazuki Hayashi  1 , Hitoshi Sano  1 , Katsuyuki Miyabe  1 , Shuya Shimizu  1 , Takashi Joh  1
Affiliations
Review

Inflammatory bowel disease of primary sclerosing cholangitis: a distinct entity?

Takahiro Nakazawa et al. World J Gastroenterol. .

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

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Figures

Figure 1
Figure 1
Colonoscopic findings at clinical onset. A: Cecum; B: Ascending colon; C: Sigmoid colon; D: Rectum. A 43-year-old female patient diagnosed with asymptomatic, concurrent primary sclerosing cholangitis-inflammatory bowel disease. The first colonoscopy showed multiple white scars in the ascending colon and right-sided transverse colon and no abnormal findings in the left-sided transverse colon, descending colon, sigmoid colon, or rectum.
Figure 2
Figure 2
Colonoscopic findings seven months later. A: Cecum; B: Ascending colon; C: Sigmoid colon; D: Rectum. A repeat colonoscopy seven months later showing inflamed mucosa with multiple erosions and redness from the ascending colon to the right-sided transverse colon. Mucosal vessels are clearly visible in the descending colon, sigmoid colon, and rectum.
Figure 3
Figure 3
Cholangiographic examples of immunoglobulin G4-related sclerosing cholangitis and primary sclerosing cholangitis. Cholangiograms of immunoglobulin G4-related sclerosing cholangitis showing multiple stenoses in the intrahepatic ducts and stenosis in the intrapancreatic portion (A, B). Cholangiograms of primary sclerosing cholangitis showing a beaded appearance (C) and pruning of the intrahepatic ducts (C, D).
Figure 4
Figure 4
Correlation between inflammatory bowel disease and screrosing cholangitis. PSC is frequently associated with characteristic PSC-IBD, whereas IgG4-SC is not associated with IBD. IgG4-SC is frequently associated with type 1 AIP, whereas type 2 AIP is frequently associated with IBD. PSC: Primary sclerosing cholangitis; PSC-IBD: IBD-associated with PSC; IBD: Inflammatory bowel disease; IgG4-SC: Immunoglobulin G4-related sclerosing cholangitis; AIP: Autoimmune pancreatitis.
See this image and copyright information in PMC

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