Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

J Hepatol. 2002 May;36(5):586-9. doi: 10.1016/s0168-8278(02)00036-3.


Background/aims: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antimetabolites / therapeutic use
  • Azathioprine / therapeutic use
  • Bile Duct Neoplasms / epidemiology
  • Bile Ducts, Extrahepatic
  • Bile Ducts, Intrahepatic
  • Cholangiocarcinoma / epidemiology
  • Cholangitis, Sclerosing / diagnosis*
  • Cholangitis, Sclerosing / drug therapy
  • Cholangitis, Sclerosing / epidemiology*
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Risk Factors
  • Steroids / therapeutic use
  • Sweden / epidemiology
  • Treatment Outcome


  • Antimetabolites
  • Steroids
  • Azathioprine