title: Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer.
abstract: OBJECTIVE
  Primary sclerosing cholangitis is a cholestatic liver disease characterized by multifocal strictures in the intra- and extrahepatic biliary tree. Dominant strictures may arise in the extrahepatic bile ducts, and in these circumstances, endoscopic therapy has been introduced to relieve cholestasis and perhaps also delay the development of liver cirrhosis. The experience of endoscopic treatment at this point in time is limited and the long-term benefit is not clear. Neoplastic transformation in primary sclerosing cholangitis is unpredictable, which is illustrated in the present study along with an evaluation of the efficacy of endoscopic treatment.
  
  
  METHODS
  Endoscopic retrograde cholangiopancreatography was performed in 25 patients with primary sclerosing cholangitis. In 15 there were dominant strictures in the hilum of the liver and/or the distal bile duct and these patients were treated by dilation and/or endoprostheses. Four patients in the treatment group had just cholestatic biochemical test results and 11 were symptomatic.
  
  
  RESULTS
  Endoscopic therapy was technically successful in all 15 patients. In 43 sessions, 5 patients were treated by dilation, 2 with endoprostheses, and 8 by both methods. Improvement was achieved radiologically in 12 patients, clinically in 8, and according to liver function tests in 7. Therapy was complicated by cholangitis in 5 patients. Complications were mild and there was no mortality related to the procedure. However, 6 patients in the treatment group died, 5 of cholangiocarcinoma and 1 of colon cancer.
  
  
  CONCLUSIONS
  Endoscopic therapy in primary sclerosing cholangitis is indicated in selected patients. The cancer incidence is high, not least in patients with deteriorating disease. It is important to find techniques for identifying patients at risk in order to perform liver transplantation before malignant transformation.
authors.affiliation: Department of Surgery, Stockholm Söder Hospital, Karolinska Institute, S-118 83 Stockholm, Sweden. stefan.linder@kirurg.sos.sll.se
authors.forename: S
authors.initials: S
authors.lastname: Linder
authors.forename: C
authors.initials: C
authors.lastname: Söderlund
mesh_terms: D000328:Adult; D000368:Aged; D001650:Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis; D001653:Bile Ducts, Intrahepatic; D002404:Catheterization; D018281:Cholangiocarcinoma / Q000150:complications / Q000175:diagnosis; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D003251:Constriction, Pathologic; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D015607:Stents