Factors Associated With Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System.

Goode, Elizabeth C and Clark, Allan B and Mells, George F and Srivastava, Brijesh and Spiess, Kelly and Gelson, William T H and Trivedi, Palak J and Lynch, Kate D and Castren, Edit and Vesterhus, Mette N and Karlsen, Tom H and Ji, Sun-Gou and Anderson, Carl A and Thorburn, Douglas and Hudson, Mark and Heneghan, Michael A and Aldersley, Mark A and Bathgate, Andrew and Sandford, Richard N and Alexander, Graeme J and Chapman, Roger W and Walmsley, Martine and Hirschfield, Gideon M and Rushbrook, Simon M (2019) Factors Associated With Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System. Hepatology (Baltimore, Md.), 69 (5). pp. 2120-2135. ISSN 1527-3350. Available through your UHB Open Athens account

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Abstract

We sought to identify factors that are predictive of liver transplantation or death in patients with primary sclerosing cholangitis (PSC), and to develop and validate a contemporaneous risk score for use in a real-world clinical setting. Analyzing data from 1,001 patients recruited to the UK-PSC research cohort, we evaluated clinical variables for their association with 2-year and 10-year outcome through Cox-proportional hazards and C-statistic analyses. We generated risk scores for short-term and long-term outcome prediction, validating their use in two independent cohorts totaling 451 patients. Thirty-six percent of the derivation cohort were transplanted or died over a cumulative follow-up of 7,904 years. Serum alkaline phosphatase of at least 2.4 × upper limit of normal at 1 year after diagnosis was predictive of 10-year outcome (hazard ratio [HR] = 3.05; C = 0.63; median transplant-free survival 63 versus 108 months; P < 0.0001), as was the presence of extrahepatic biliary disease (HR = 1.45; P = 0.01). We developed two risk scoring systems based on age, values of bilirubin, alkaline phosphatase, albumin, platelets, presence of extrahepatic biliary disease, and variceal hemorrhage, which predicted 2-year and 10-year outcomes with good discrimination (C statistic = 0.81 and 0.80, respectively). Both UK-PSC risk scores were well-validated in our external cohort and outperformed the Mayo Clinic and aspartate aminotransferase-to-platelet ratio index (APRI) scores (C statistic = 0.75 and 0.63, respectively). Although heterozygosity for the previously validated human leukocyte antigen (HLA)-DR*03:01 risk allele predicted increased risk of adverse outcome (HR = 1.33; P = 0.001), its addition did not improve the predictive accuracy of the UK-PSC risk scores. Conclusion: Our analyses, based on a detailed clinical evaluation of a large representative cohort of participants with PSC, furthers our understanding of clinical risk markers and reports the development and validation of a real-world scoring system to identify those patients most likely to die or require liver transplantation.

Item Type: Article
Additional Information: Available through your UHB Open Athens account
Subjects: WI Digestive system. Gastroenterology
Divisions: Planned IP Care > Gastroentrology
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Depositing User: Beth Connors
Date Deposited: 19 Jun 2020 13:06
Last Modified: 19 Jun 2020 13:06
URI: http://www.repository.uhblibrary.co.uk/id/eprint/3186

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