Clinical Characteristics, Associated Malignancies and Management of Primary Sclerosing Cholangitis in Inflammatory Bowel Disease Patients: A Multicentre Retrospective Cohort Study

J Crohns Colitis. 2019 Dec 10;13(12):1492-1500. doi: 10.1093/ecco-jcc/jjz094.

Abstract

Background and aims: Primary sclerosing cholangitis [PSC] is usually associated with inflammatory bowel disease [IBD]. An increased risk of malignancies, mainly colorectal cancer [CRC] and cholangiocarcinoma [CCA], has been reported in PSC-IBD patients. Our aim was to determine the clinical characteristics and management of PSC in IBD patients, and the factors associated with malignancies.

Methods: PSC-IBD patients were identified from the Spanish ENEIDA registry of GETECCU. Additional data were collected using the AEG-REDCap electronic data capture tool.

Results: In total, 277 PSC-IBD patients were included, with an incidence rate of 61 PSC cases per 100 000 IBD patient-years, 69.7% men, 67.5% ulcerative colitis and mean age at PSC diagnosis of 40 ± 16 years. Most patients [85.2%] were treated with ursodeoxycholic acid. Liver transplantation was required in 35 patients [12.6%] after 79 months (interquartile range [IQR] 50-139). It was more common in intra- and extrahepatic PSC compared with small-duct PSC (16.3% vs 3.3%; odds ratio [OR] 5.7: 95% confidence interval [CI] = 1.7-19.3). The incidence rate of CRC since PSC diagnosis was 3.3 cases per 1000 patient-years [95% CI = 1.9-5.6]. Having symptoms of PSC at PSC diagnosis was the only factor related to an increased risk of CRC after IBD diagnosis [hazard ratio= 3.3: 95% CI = 1.1-9.9]. CCA was detected in seven patients [2.5%] with intra- and extrahepatic PSC, with median age of 42 years [IQR 39-53], and presented a lower life expectancy compared with patients without CCA and patients with or without CRC.

Conclusions: PSC-IBD patients with symptoms of PSC at PSC diagnosis have an increased risk of CRC. CCA was only diagnosed in patients with intra- and extrahepatic PSC and was associated with poor survival.

Keywords: Primary sclerosing cholangitis; inflammatory bowel disease; malignancy.

Publication types

  • Multicenter Study
  • Observational Study

MeSH terms

  • Adult
  • Bile Ducts, Extrahepatic / pathology
  • Bile Ducts, Intrahepatic / pathology
  • Cholangiocarcinoma* / diagnosis
  • Cholangiocarcinoma* / mortality
  • Cholangitis, Sclerosing* / diagnosis
  • Cholangitis, Sclerosing* / epidemiology
  • Cholangitis, Sclerosing* / physiopathology
  • Cholangitis, Sclerosing* / therapy
  • Colorectal Neoplasms* / diagnosis
  • Colorectal Neoplasms* / mortality
  • Female
  • Humans
  • Inflammatory Bowel Diseases* / diagnosis
  • Inflammatory Bowel Diseases* / epidemiology
  • Inflammatory Bowel Diseases* / physiopathology
  • Inflammatory Bowel Diseases* / therapy
  • Male
  • Middle Aged
  • Patient Care Management / methods
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Spain / epidemiology
  • Survival Analysis