Tyck till om SwePub Sök
här!
Search: WFRF:(Chapman Roger W)
> Bergquist Annika >
The natural history...
The natural history of small duct primary sclerosing cholangitis
-
Björnsson, Einar (author)
-
Olsson, Rolf (author)
-
- Bergquist, Annika (author)
- Karolinska Institutet
-
show more...
-
- Lindgren, Stefan (author)
- Lund University,Lunds universitet,Enheten för kroniska inflammatoriska och degenerativa sjukdomar,Forskargrupper vid Lunds universitet,Chronic Inflammatory and Degenerative Diseases Research Unit,Lund University Research Groups
-
Braden, Barbara (author)
-
Chapman, Roger W (author)
-
Boberg, Kirsten M (author)
-
Angulo, Paul (author)
-
show less...
-
(creator_code:org_t)
- Elsevier BV, 2008
- 2008
- English.
-
In: Gastroenterology. - : Elsevier BV. - 1528-0012 .- 0016-5085. ; 134:4, s. 975-980
- Related links:
-
http://dx.doi.org/10...
-
show more...
-
https://lup.lub.lu.s...
-
https://doi.org/10.1...
-
http://kipublication...
-
show less...
Abstract
Subject headings
Close
- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Gastroenterologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Gastroenterology and Hepatology (hsv//eng)
Keyword
- MRC
- magnetic resonance cholangiography
- PSC
- primary sclerosing cholangitis
- ursodeoxycholic acid
- UDCA
- interquartile range
- IQR
Publication and Content Type
- art (subject category)
- ref (subject category)
Find in a library
To the university's database