title: Appearance of hepatobiliary diseases in a population-based cohort with inflammatory bowel diseases (Inflammatory Bowel Disease Cohort of the Uppsala Region).
abstract: OBJECTIVE
  To prospectively follow the evolution of hepatobiliary diseases in a population-based cohort of patients with inflammatory bowel diseases.
  
  
  METHODS
  Between 2005 and 2009, 790 incident cases of ulcerative colitis and Crohn's disease were registered in the Uppsala Health Region, corresponding to an average incidence of 20.0 and 9.9 new cases/100 000 inhabitants/year, respectively. Liver function tests were analyzed in 97.1% and the results of ensuing investigations were summarized.
  
  
  RESULTS
  Seventeen patients with primary sclerosing cholangitis were diagnosed corresponding to an overall prevalence of 2.2% (ulcerative colitis 1.7% and Crohn's disease 3.0%, respectively). The median age at diagnosis was 25 years (interquartile range: 17.0-34.0). Among the 92 patients below 17 years of age, three had autoimmune hepatitis and three primary sclerosing cholangitis, summing up to a prevalence of 6.5% immune-mediated hepatobiliary diseases among the pediatric patients. Three patients have undergone liver transplantation and one died of colonic carcinoma. Ten patients have demonstrated persistent elevation of alkaline phosphatases but had a normal magnetic resonance cholangiopancreatography (two patients) or refused further investigation (one patient).
  
  
  CONCLUSIONS
  In this first large prospective population-based cohort of 526 patients with ulcerative colitis (UC) and 264 with Crohn's disease, 17 cases of primary sclerosing cholangitis were found, among whom three (17%) so far have been liver transplanted and one has died of colon carcinoma. The average age of those affected by primary sclerosing cholangitis is considerably lower than usually reported. Ten patients had or have had elevated alkaline phosphatase without confirmed liver or biliary disease.
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Rönnblom
authors.affiliation: Department of Internal Medicine, Mariehamn, Åland, Finland.
authors.forename: Tommy
authors.initials: T
authors.lastname: Holmström
authors.affiliation: Department of Internal Medicine, Mälar Hospital, Eskilstuna, Sweden.
authors.forename: Hans
authors.initials: H
authors.lastname: Tanghöj
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
authors.forename: Fredrik
authors.initials: F
authors.lastname: Rorsman
authors.affiliation: Department of Internal Medicine, Falu Hospital, Falun, Sweden.
authors.forename: Daniel
authors.initials: D
authors.lastname: Sjöberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000469:Alkaline Phosphatase / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002648:Child; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D015331:Cohort Studies; D003093:Colitis, Ulcerative / Q000453:epidemiology*; D015897:Comorbidity; D003424:Crohn Disease / Q000453:epidemiology*; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D015996:Survival Rate; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult
chemical_list: D015415:Biomarkers; D000469:Alkaline Phosphatase
keywords: autoimmune hepatitis; hepatobiliary diseases; inflammatory bowel diseases; primary sclerosing cholangitis