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
. 2005 Jan;54(1):91-6.
doi: 10.1136/gut.2004.046615.

PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis

E V Loftus Jr  1 , G C Harewood, C G Loftus, W J Tremaine, W S Harmsen, A R Zinsmeister, D A Jewell, W J Sandborn
Affiliations

PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis

E V Loftus Jr et al. Gut. 2005 Jan.

Abstract

Background: Inflammatory bowel disease associated with primary sclerosing cholangitis (PSC-IBD) may have a high prevalence of rectal sparing, backwash ileitis, and colorectal neoplasia.

Aims: To describe the clinical features and outcomes of PSC-IBD and compare these to a group of chronic ulcerative colitis (CUC) patients.

Methods: The medical records of all patients with PSC-IBD evaluated at the Mayo Clinic Rochester between 1987 and 1992 were abstracted for information on endoscopic and histological features, colorectal neoplasia, surgery, and other clinical outcomes. Patients referred for colorectal neoplasia and those who did not undergo colonoscopy with biopsies were excluded. A control group of CUC patients matched for sex, duration of IBD at first clinic visit, and calendar year of first clinic visit was identified, and similar information was abstracted.

Results: Seventy one PSC-IBD patients and 142 CUC patients without PSC were identified. Rectal sparing and backwash ileitis were more common in the PSC-IBD group (52% and 51%, respectively) than in controls (6% and 7%, respectively). Overall, colorectal neoplasia developed in 18 cases and 15 controls, including 11 cancers (seven cases and four controls). An increased risk of colorectal neoplasia or death was not detected in a matched analysis. Although the cumulative incidence of colorectal neoplasia was higher in cases (33%) than in controls (13%) at five years, this was of borderline statistical significance (p=0.054, unmatched log rank test). Overall survival from first clinic visit was significantly worse among cases (79% v 97%) at five years (p<0.001, unmatched log rank test).

Conclusion: PSC-IBD is frequently characterised by rectal sparing and backwash ileitis. Colorectal neoplasia develops in a substantial fraction and overall survival is worse. PSC-IBD may represent a distinct IBD phenotype.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Cumulative probability (1−survival free) of colorectal cancer or dysplasia from the observational start date in cases (those with inflammatory bowel disease associated with primary sclerosing cholangitis) versus controls (those with chronic ulcerative colitis).
Figure 2
Figure 2
Overall survival from observational start date in cases (those with inflammatory bowel disease associated with primary sclerosing cholangitis) versus controls (those with chronic ulcerative colitis). (Follow up was censored for those who underwent orthotopic liver transplantation.)
See this image and copyright information in PMC

References

    1. Wiesner RH, LaRusso NF. Clinicopathologic features of the syndrome of primary sclerosing cholangitis. Gastroenterology 1980;79:200–6. - PubMed
    1. Chapman RW, Arborgh BA, Rhodes JM, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology. Gut 1980;21:870–7. - PMC - PubMed
    1. LaRusso NF, Wiesner RH, Ludwig J, et al. Current concepts. Primary sclerosing cholangitis. N Engl J Med 1984;310:899–903. - PubMed
    1. Wee A , Ludwig J, Coffey RJ Jr, et al. Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis. Hum Pathol 1985;16:719–26. - PubMed
    1. Rosen CB, Nagorney DM, Wiesner RH, et al. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg 1991;213:21–5. - PMC - PubMed