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The natural history of small duct primary sclerosing cholangitis

Björnsson, Einar; Olsson, Rolf; Bergquist, Annika; Lindgren, Stefan LU ; Braden, Barbara; Chapman, Roger W; Boberg, Kirsten M and Angulo, Paul (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)
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author
organization
publishing date
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
W B Saunders
external identifiers
  • wos:000254853800017
  • scopus:41349103851
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
2008-04-24 15:44:41
date last changed
2017-11-05 03:38:48
@article{c6a112ec-ed39-451e-9d35-0e69b0128f95,
  abstract     = {Background &amp; 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 &lt; .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},
  keyword      = {MRC,magnetic resonance cholangiography,PSC,primary sclerosing cholangitis,ursodeoxycholic acid,UDCA,interquartile range,IQR},
  language     = {eng},
  number       = {4},
  pages        = {975--980},
  publisher    = {W B Saunders},
  series       = {Gastroenterology},
  title        = {The natural history of small duct primary sclerosing cholangitis},
  url          = {http://dx.doi.org/10.1053/j.gastro.2008.01.042},
  volume       = {134},
  year         = {2008},
}