Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Apr;1(1):9-14.
doi: 10.1159/000445259. Epub 2016 Apr 1.

Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?

Atsushi Tanaka  1 , Joachim C Mertens  2
Affiliations
Review

Ulcerative Colitis with and without Primary Sclerosing Cholangitis: Two Different Diseases?

Atsushi Tanaka et al. Inflamm Intest Dis. 2016 Apr.

Abstract

Background: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin and an important hepatobiliary complication of inflammatory bowel diseases (IBD), especially ulcerative colitis (UC). When further differentiated, about 3-8% of UC patients suffer from PSC, whereas among Crohn's disease patients the reported prevalence of PSC is probably between 1 and 3.5%. Although it was reported from Japan that the frequency of PSC in UC was only 34%, the same registry data indicated that the prevalence was up to 57% among young patients with PSC even in Japan, which is comparable to the 60-80% in Europe and the US. Additionally, the clinical features of UC in patients with PSC are different from those in patients without PSC, for instance, rectal sparing and right-sided dominance.

Summary: The strong link between atypical IBD and PSC suggests that the pathogenesis of PSC involves pathology of the gut, including abnormal gut microbiota and aberrant activation of mucosal lymphocytes. These seem to be different in UC PSC as compared to the pathology of typical UC.

Key messages: The key to solving the question 'Is there a difference between East and West?' are genetic studies, genome-wide association studies of PSC in particular, which have already been performed in the West and are strongly warranted in the East.

Keywords: Primary sclerosing cholangitis; Ulcerative colitis.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Age distribution at presentation of patients with PSC in Japan.
Fig. 2
Fig. 2
Prevalence of IBD in patients with PSC in Japan. CF = Colonofiberscope.
See this image and copyright information in PMC

References

    1. Karlsen TH, Boberg KM. Update on primary sclerosing cholangitis. J Hepatol. 2013;59:571–582. - PubMed
    1. Mendes FD, et al. Abnormal hepatic biochemistries in patients with inflammatory bowel disease. Am J Gastroenterol. 2007;102:344–350. - PubMed
    1. Fausa O, Schrumpf E, Elgjo K. Relationship of inflammatory bowel disease and primary sclerosing cholangitis. Semin Liver Dis. 1991;11:31–39. - PubMed
    1. Olsson R, et al. Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastroenterology. 1991;100:1319–1323. - PubMed
    1. Saich R, Chapman R. Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J Gastroenterol. 2008;14:331–337. - PMC - PubMed

LinkOut - more resources