title: Patients with large-duct primary sclerosing cholangitis and Crohn's disease have a better outcome than those with ulcerative colitis, or without IBD.
abstract: BACKGROUND
  Approximately 20% of primary sclerosing cholangitis (PSC) patients with concomitant inflammatory bowel disease (IBD) have Crohn's disease (CD).
  
  
  OBJECTIVE
  To compare PSC/CD with other PSC patients.
  
  
  METHODS
  Retrospective study of 240 PSC patients diagnosed between 1975 and 2012 (median follow-up 12 years). Activity of PSC at diagnosis was assessed by liver biopsy, Mayo risk and ERC scores. Survival without liver transplantation, number of transplantations and liver-related death were endpoints.
  
  
  RESULTS
  Sixty-three per cent of patients had IBD: 105 UC, 32 CD and 14 IBD unclassified (IBDu). IBD was diagnosed before PSC in 50%. The yearly development of PSC after diagnosing IBD was similar in UC, CD or IBDu. Small-duct PSC was present in 28% of PSC/CD compared to 3% of PSC/UC. Small-duct PSC had a markedly better survival than large-duct PSC: no patient developed cholangiocarcinoma or liver-related death, but colorectal cancer occurred in three patients. In large-duct PSC, a more favourable outcome was evident in patients with CD. The liver disease was less progressive: one patient underwent liver transplantation compared to 28% and liver-related deaths were absent compared to 7% in the other PSC groups.
  
  
  CONCLUSIONS
  The prevalence of PSC with concomitant Crohn's disease is relatively rare, but the outcome is more benign than PSC with UC or without IBD. Approximately one-fourth has small-duct PSC. In large-duct PSC/CD, liver disease is less aggressive and the outcome is much better. The outcome of PSC patients with UC resembled that of PSC without IBD.
authors.affiliation: Hepatology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Fevery
authors.affiliation: Hepatology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: W
authors.initials: W
authors.lastname: Van Steenbergen
authors.affiliation: Hepatology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: J
authors.initials: J
authors.lastname: Van Pelt
authors.affiliation: Hepatology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: W
authors.initials: W
authors.lastname: Laleman
authors.affiliation: Paediatric Gastroenterology, Department of Clinical and Experimental Medicine, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: I
authors.initials: I
authors.lastname: Hoffman
authors.affiliation: Pathology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: K
authors.initials: K
authors.lastname: Geboes
authors.affiliation: Gastroenterology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: S
authors.initials: S
authors.lastname: Vermeire
authors.affiliation: Hepatology, University Hospital Gasthuisberg, KU Leuven, Leuven, Belgium.
authors.forename: F
authors.initials: F
authors.lastname: Nevens
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D015209:Cholangitis, Sclerosing / Q000145:classification / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D003093:Colitis, Ulcerative / Q000453:epidemiology*; D015179:Colorectal Neoplasms / Q000150:complications; D003424:Crohn Disease / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000453:epidemiology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D055815:Young Adult