The natural history of small-duct primary sclerosing cholangitis.
BACKGROUND and 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...
| Main Authors: | , , , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2008
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| _version_ | 1826306631073267712 |
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| author | Björnsson, E Olsson, R Bergquist, A Lindgren, S Braden, B Chapman, R Boberg, K Angulo, P |
| author_facet | Björnsson, E Olsson, R Bergquist, A Lindgren, S Braden, B Chapman, R Boberg, K Angulo, P |
| author_sort | Björnsson, E |
| collection | OXFORD |
| description | BACKGROUND and 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. |
| first_indexed | 2024-03-07T06:50:53Z |
| format | Journal article |
| id | oxford-uuid:fc8440c7-b612-4971-b51d-94467b50fae6 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-07T06:50:53Z |
| publishDate | 2008 |
| record_format | dspace |
| spelling | oxford-uuid:fc8440c7-b612-4971-b51d-94467b50fae62022-03-27T13:21:24ZThe natural history of small-duct primary sclerosing cholangitis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:fc8440c7-b612-4971-b51d-94467b50fae6EnglishSymplectic Elements at Oxford2008Björnsson, EOlsson, RBergquist, ALindgren, SBraden, BChapman, RBoberg, KAngulo, P BACKGROUND and 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. |
| spellingShingle | Björnsson, E Olsson, R Bergquist, A Lindgren, S Braden, B Chapman, R Boberg, K Angulo, P The natural history of small-duct primary sclerosing cholangitis. |
| title | The natural history of small-duct primary sclerosing cholangitis. |
| title_full | The natural history of small-duct primary sclerosing cholangitis. |
| title_fullStr | The natural history of small-duct primary sclerosing cholangitis. |
| title_full_unstemmed | The natural history of small-duct primary sclerosing cholangitis. |
| title_short | The natural history of small-duct primary sclerosing cholangitis. |
| title_sort | natural history of small duct primary sclerosing cholangitis |
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