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BACKGROUND & AIMS: We sought to identify factors 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. METHODS: Analysing data from 1001 patients recruited to the UK-PSC research cohort, we evaluated clinical variables for their association with 2- and 10-year outcome through Cox-proportional hazards and C-statistic analyses. We generated risk scores for short- and long-term outcome prediction, validating their use in two independent cohorts totalling 451 patients. RESULTS: 36% of the derivation cohort were transplanted or died over a cumulative follow-up of 7,904 years. Serum alkaline phosphatase ≥2.4×ULN at 1 year post diagnosis, was predictive of 10-year outcome (HR=3.05, C=0.63, median transplant-free survival 63 versus 108 months, p<0.0001), as was the presence of extra-hepatic biliary disease (HR=1.45, p=0.01). We developed two risk scoring systems based upon age, values of bilirubin, alkaline phosphatase, albumin, platelets, presence of extra-hepatic biliary disease and variceal haemorrhage, which predicted 2- and 10-year outcome with good discrimination (C=0.81 and 0.80 respectively). Both UK-PSC risk scores were well-validated in our external cohort, and out-performed the Mayo and APRI scores (C=0.75 and 0.63 respectively). Whilst heterozygosity for the previously validated HLA-DR*03:01 risk allele predicted increased risk of adverse outcome (HR=1.33, p=.001), its addition did not improve the predictive accuracy the UK-PSC risk scores. CONCLUSIONS: Our analyses, based upon 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. This article is protected by copyright. All rights reserved.

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UK-PSC, autoimmune liver disease, cholestasis, prognostic factor, risk score