title: Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC).
abstract: OBJECTIVE
  This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC).
  
  
  METHODS
  Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1-168) months. The median follow-up time was 63 (1-194) months.
  
  
  RESULTS
  All patients including one who underwent liver transplantation because of end-stage liver disease and hepatocellular carcinoma were alive at follow-up. None developed cholangiocarcinoma. In 27 patients repeated cholangiographic examinations were done after a median time of 72 (12-192) months from first ERCP. Four developed features of large duct PSC.
  
  
  CONCLUSIONS
  Small duct PSC rarely progresses to large bile duct PSC and it seems to have a benign course in most patients and no development of cholangiocarcinoma was found.
authors: Department of Gastroenterology, Huddinge University Hospital, Stockholm, Sweden. ulrika.broome@medhs.ki.se
authors: Ulrika
authors: U
authors: Broomé
authors: Hans
authors: H
authors: Glaumann
authors: Eva
authors: E
authors: Lindstöm
authors: Lars
authors: L
authors: Lööf
authors: Sven
authors: S
authors: Almer
authors: Hanne
authors: H
authors: Prytz
authors: Hanna
authors: H
authors: Sandberg-Gertzén
authors: Stefan
authors: S
authors: Lindgren
authors: Frans-Thomas
authors: FT
authors: Fork
authors: Gunnar
authors: G
authors: Järnerot
authors: Rolf
authors: R
authors: Olsson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000963:Antimetabolites / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D001650:Bile Duct Neoplasms / Q000453:epidemiology; D017734:Bile Ducts, Extrahepatic; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000453:epidemiology; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012307:Risk Factors; D013256:Steroids / Q000627:therapeutic use; D013548:Sweden / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D000963:Antimetabolites; D013256:Steroids; D001379:Azathioprine