Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2002 Nov;51(5):731-5.
doi: 10.1136/gut.51.5.731.

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

E Björnsson  1 , K M Boberg, S Cullen, K Fleming, O P Clausen, O Fausa, E Schrumpf, R W Chapman
Affiliations

Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis

E Björnsson et al. Gut. 2002 Nov.

Abstract

Background: Patients with cholestatic liver function tests and histological features of primary sclerosing cholangitis (PSC) but a normal cholangiogram are considered to have small duct PSC. The natural history of this condition is unknown.

Methods: Thirty three patients with small duct PSC were identified among patients admitted for diagnostic workup of cholestatic liver function tests in one centre in the UK (Oxford) and one centre in Norway (Oslo). A total of 260 patients with large duct PSC were compared, and prognosis in terms of death, cholangiocarcinoma, biochemical features, histological features, and symptoms analysed.

Results: Mean age at diagnosis was 38 years and 39 years in small duct and large duct PSC, respectively. Mean follow up was 106 months in small duct and 105 months in large duct patients. Four patients originally considered to have small duct developed large duct PSC. Two of these underwent liver transplantation during follow up. Of the remainder who did not develop large duct PSC, two patients died during follow up: one of liver failure and the other of cardiac death unrelated to her liver disease. A total of 122 (47%) large duct patients either required liver transplantation (34 patients) or died (88 patients). Small duct patients had a significantly better survival compared with large duct patients. Among small duct patients, none developed cholangiocarcinoma compared with 28 of 260 (11%) large duct patients.

Conclusions: Patients with small duct PSC seem to have a good prognosis in terms of survival and development of cholangiocarcinoma. Small duct PSC progresses to large duct PSC in a small proportion of patients.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Kaplan-Meier estimated survival curves for patients with small duct and large duct primary sclerosing cholangitis (p<0.01).
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Ludwig J. Histopathology of primary sclerosing cholangitis. In: Manns M, Chapman RW, Stiehl A, et al, eds. Primary sclerosing cholangitis. London: Kluwer Academic publishers, 1998:14–21.
    1. Chapman RW, Arborgh BM, Rhodes JM, et al. Primary sclerosing cholangitis: a review of its clinical features, cholangiography and hepatic histology. Gut 1980;21:1870–7. - PMC - PubMed
    1. Broomé U, Olsson R, Lööf L, et al. Natural history and prognostic factors in 305 patients with primary sclerosing cholangitis. Gut 1996;38:610–15. - PMC - PubMed
    1. Wiesner TH, Grambsch PB, Dickson ER, et al. Primary sclerosing cholangitis: natural history, prognostic factors, and survival analysis. Hepatology 1989;10:430–6. - PubMed
    1. Farrant JM, Hayllar KM, Wilkinson ML, et al. Natural history and prognostic variables in primary sclerosing cholangitis. Gastroenterology 1991;100:1710–17. - PubMed

Publication types