title: Primary sclerosing cholangitis associated with inflammatory bowel disease: an observational study in a Southern Europe population focusing on new therapeutic options.
abstract: BACKGROUND
  Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with a strong association with inflammatory bowel disease (IBD). Medical treatment for PSC is still disappointing, whereas immunomodulators and biologics have been proven to be effective in IBD.
  
  
  OBJECTIVE
  This study aimed to analyze (i) the natural history of patients with PSC with or without IBD and (ii) the long-term efficacy of biologics in patients with PSC and concomitant IBD or rheumatological disorders.
  
  
  METHODS
  This study included 92 consecutive PSC patients, 50 (54.3%) men and 42 (45.7%) women, with a mean age of 32.0±14.3 years at diagnosis and a mean follow-up duration of 103.8±86 months. Forty-nine (53.3%) patients had associated IBD (38 ulcerative colitis, 10 Crohn's disease, one indeterminate colitis).
  
  
  RESULTS
  No significant differences were found between PSC patients with and without associated IBD in terms of liver transplantation, cancer, and death rates. Cholangiocarcinoma was only identified among patients with PSC alone, whereas other cancers (hepatocellular carcinoma, colorectal, and gallbladder cancer) were found only in the group with associated IBD. Five PSC patients were treated with biologic agents: three with adalimumab and one with infliximab for IBD or for rheumatoid arthritis, and one patient with rituximab for rheumatoid arthritis. Adalimumab decreased alkaline phosphatase in two of three patients after 6 and 12 months, infliximab reduced γ-glutamyltransferase after 6 and 12 months, but liver function tests tended to deteriorate thereafter. Cholangiography changes remained stable in all patients.
  
  
  CONCLUSIONS
  Biologic agents may improve liver function tests in PSC patients, but may be associated with adverse events including deterioration of liver function.
authors.affiliation: aDepartment of Surgery, Oncology and Gastroenterology bDepartment of Medicine cLaboratory of Public Health and Population Studies, Department of Molecular Medicine, University of Padova, Padova, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Franceschet
authors.forename: Nora
authors.initials: N
authors.lastname: Cazzagon
authors.forename: Teresa
authors.initials: T
authors.lastname: Del Ross
authors.forename: Renata
authors.initials: R
authors.lastname: D'Incà
authors.forename: Alessandra
authors.initials: A
authors.lastname: Buja
authors.forename: Annarosa
authors.initials: A
authors.lastname: Floreani
mesh_terms: D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001650:Bile Duct Neoplasms / Q000453:epidemiology; D001688:Biological Products / Q000009:adverse effects / Q000627:therapeutic use*; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology; D018281:Cholangiocarcinoma / Q000453:epidemiology; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000453:epidemiology; D003093:Colitis, Ulcerative / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D015179:Colorectal Neoplasms / Q000453:epidemiology; D003424:Crohn Disease / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D018450:Disease Progression; D005060:Europe / Q000453:epidemiology; D005260:Female; D005706:Gallbladder Neoplasms / Q000453:epidemiology; D005765:Gastrointestinal Agents / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D008111:Liver Function Tests; D008113:Liver Neoplasms / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D001688:Biological Products; D005765:Gastrointestinal Agents