The natural history of small duct primary sclerosing cholangitis
(2008) In Gastroenterology 134(4). p.975-980- Abstract
- Background & Aims: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. Methods: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United States were combined. Each patient with small-duct PSC was randomly matched to 2 patients with large-duct PSC by age, gender, calendar year of diagnosis, and institution. Results: The median age at diagnosis in both groups was 38 years (61% males). Nineteen (22.9%) of the 83 patients with small-duct PSC progressed to large-duct PSC in a median of 7.4... (More)
- Background & Aims: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. Methods: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United States were combined. Each patient with small-duct PSC was randomly matched to 2 patients with large-duct PSC by age, gender, calendar year of diagnosis, and institution. Results: The median age at diagnosis in both groups was 38 years (61% males). Nineteen (22.9%) of the 83 patients with small-duct PSC progressed to large-duct PSC in a median of 7.4 (interquartile range [IQR], 5.1–14) years. One patient with small-duct PSC who progressed to large-duct PSC was diagnosed with cholangiocarcinoma but after progression to large-duct PSC; 20 patients with large-duct PSC developed cholangiocarcinoma. Patients with small-duct PSC had a significantly longer transplantation-free survival compared with large-duct PSC patients (13 years [IQR, 10–17] vs 10 years [IQR, 6–14], respectively; hazard ratio, 3.04; 95% confidence interval: 1.82–5.06; P < .0001). Two patients with small-duct PSC who underwent liver transplantation had recurrence of small-duct PSC in the graft 9 and 13 years, respectively, after transplantation. Conclusions: Small-duct PSC is a disease of progressive potential but associated with a better long-term prognosis as compared with large-duct PSC. Small-duct PSC may recur after liver transplantation. Cholangiocarcinoma does not seem to occur in patients with small-duct PSC, unless the disease has progressed to large-duct PSC. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1144378
- author
- Björnsson, Einar ; Olsson, Rolf ; Bergquist, Annika ; Lindgren, Stefan LU ; Braden, Barbara ; Chapman, Roger W ; Boberg, Kirsten M and Angulo, Paul
- organization
- publishing date
- 2008
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- MRC, magnetic resonance cholangiography, PSC, primary sclerosing cholangitis, ursodeoxycholic acid, UDCA, interquartile range, IQR
- in
- Gastroenterology
- volume
- 134
- issue
- 4
- pages
- 975 - 980
- publisher
- Elsevier
- external identifiers
-
- wos:000254853800017
- scopus:41349103851
- pmid:18395078
- ISSN
- 1528-0012
- DOI
- 10.1053/j.gastro.2008.01.042
- language
- English
- LU publication?
- yes
- id
- c6a112ec-ed39-451e-9d35-0e69b0128f95 (old id 1144378)
- date added to LUP
- 2016-04-01 12:05:57
- date last changed
- 2022-04-21 02:25:47
@article{c6a112ec-ed39-451e-9d35-0e69b0128f95,
abstract = {{Background & Aims: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. Methods: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United States were combined. Each patient with small-duct PSC was randomly matched to 2 patients with large-duct PSC by age, gender, calendar year of diagnosis, and institution. Results: The median age at diagnosis in both groups was 38 years (61% males). Nineteen (22.9%) of the 83 patients with small-duct PSC progressed to large-duct PSC in a median of 7.4 (interquartile range [IQR], 5.1–14) years. One patient with small-duct PSC who progressed to large-duct PSC was diagnosed with cholangiocarcinoma but after progression to large-duct PSC; 20 patients with large-duct PSC developed cholangiocarcinoma. Patients with small-duct PSC had a significantly longer transplantation-free survival compared with large-duct PSC patients (13 years [IQR, 10–17] vs 10 years [IQR, 6–14], respectively; hazard ratio, 3.04; 95% confidence interval: 1.82–5.06; P < .0001). Two patients with small-duct PSC who underwent liver transplantation had recurrence of small-duct PSC in the graft 9 and 13 years, respectively, after transplantation. Conclusions: Small-duct PSC is a disease of progressive potential but associated with a better long-term prognosis as compared with large-duct PSC. Small-duct PSC may recur after liver transplantation. Cholangiocarcinoma does not seem to occur in patients with small-duct PSC, unless the disease has progressed to large-duct PSC.}},
author = {{Björnsson, Einar and Olsson, Rolf and Bergquist, Annika and Lindgren, Stefan and Braden, Barbara and Chapman, Roger W and Boberg, Kirsten M and Angulo, Paul}},
issn = {{1528-0012}},
keywords = {{MRC; magnetic resonance cholangiography; PSC; primary sclerosing cholangitis; ursodeoxycholic acid; UDCA; interquartile range; IQR}},
language = {{eng}},
number = {{4}},
pages = {{975--980}},
publisher = {{Elsevier}},
series = {{Gastroenterology}},
title = {{The natural history of small duct primary sclerosing cholangitis}},
url = {{http://dx.doi.org/10.1053/j.gastro.2008.01.042}},
doi = {{10.1053/j.gastro.2008.01.042}},
volume = {{134}},
year = {{2008}},
}