PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitis
- E V Loftus Jr1,
- G C Harewood1,
- C G Loftus1,
- W J Tremaine1,
- W S Harmsen2,
- A R Zinsmeister2,
- D A Jewell1,
- W J Sandborn1
- 1Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN, USA
- 2Division of Biostatistics, Mayo Clinic and Foundation, Rochester, MN, USA
- Correspondence to:
Dr E V Loftus Jr,
Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA;
- Accepted 22 June 2004
- Revised 18 June 2004
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.
- PSC-IBD, inflammatory bowel disease associated with primary sclerosing cholangitis
- CUC, chronic ulcerative colitis
- OLT, orthotopic liver transplantation
- CD, Crohn’s disease
- IC, indeterminate colitis
- PHREG, proportional hazards regression
- HR, hazard ratio
- primary sclerosing cholangitis
- chronic ulcerative colitis
- colorectal cancer
- colorectal dysplasia
- inflammatory bowel disease
Conflict of interest: None declared.
Presented in part at the 100th Annual Meeting of the American Gastroenterological Association, Orlando, Florida, May 1999 (Gastroenterology 1999;116:A732) and at the 64th Annual Meeting of the American College of Gastroenterology, Phoenix, Arizona, October 1999 (Am J Gastroenterol 1999;94:2658).