Skip to main page content
Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
, 12 (1), 17-29

Inflammatory Bowel Disease and Primary Sclerosing Cholangitis: A Review of the Phenotype and Associated Specific Features

Affiliations
Review

Inflammatory Bowel Disease and Primary Sclerosing Cholangitis: A Review of the Phenotype and Associated Specific Features

Carolina Palmela et al. Gut Liver.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic disease that is associated with inflammatory bowel disease (IBD) in approximately 70% of cases. Although the pathogenesis is still unknown for both diseases, there is increasing evidence to indicate that they share a common underlying predisposition. Herein, we review the epidemiology, diagnosis, disease pathogenesis, and specific clinical features of the PSC-IBD phenotype. Patients with PSC-IBD have a distinct IBD phenotype with an increased incidence of pancolitis, backwash ileitis, and rectal sparing. Despite often having extensive colonic involvement, these patients present with mild intestinal symptoms or are even asymptomatic, which can delay the diagnosis of IBD. Although the IBD phenotype has been well characterized in PSC patients, the natural history and disease behavior of PSC in PSC-IBD patients is less well defined. There is conflicting evidence regarding the course of IBD in PSC-IBD patients who receive liver transplantation and their risk of recurrent PSC. IBD may also be associated with an increased risk of cholangiocarcinoma in PSC patients. Overall, the PSC-IBD population has an increased risk of developing colorectal neoplasia compared to the conventional IBD population. Lifelong annual surveillance colonoscopy is currently recommended.

Keywords: Cholangitis, sclerosing; Colorectal neoplasms; Diagnosis; Inflammatory bowel disease; Liver transplantation.

Conflict of interest statement

CONFLICTS OF INTEREST

No potential conflict of interest relevant to this article was reported.

Figures

Fig. 1
Fig. 1
Possible hypothesis linking primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) pathogenesis, including the genetic predisposition, immune-mediated processes, altered gut microbiota and altered bile acid (BA) metabolism. GWAS, genome-wide association studies.
Fig. 2
Fig. 2
Phenotypic features of primary sclerosing cholangitis and inflammatory bowel disease.

Similar articles

See all similar articles

Cited by 11 PubMed Central articles

See all "Cited by" articles

References

    1. Harbord M, Annese V, Vavricka SR, et al. The first European evidence-based consensus on extra-intestinal manifestations in inflammatory bowel disease. J Crohns Colitis. 2016;10:239–254. doi: 10.1093/ecco-jcc/jjv213. - DOI - PMC - PubMed
    1. Smith MP, Loe RH. Sclerosing cholangitis; review of recent case reports and associated diseases and four new cases. Am J Surg. 1965;110:239–246. doi: 10.1016/0002-9610(65)90018-8. - DOI - PubMed
    1. Rossi RE, Conte D, Massironi S. Primary sclerosing cholangitis associated with inflammatory bowel disease: an update. Eur J Gastroenterol Hepatol. 2016;28:123–131. - PubMed
    1. Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol. 1998;33:99–103. doi: 10.1080/00365529850166284. - DOI - PubMed
    1. Molodecky NA, Kareemi H, Parab R, et al. Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis. Hepatology. 2011;53:1590–1599. doi: 10.1002/hep.24247. - DOI - PubMed

LinkOut - more resources

Feedback