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This study aimed to validate autotaxin as a biomarker in two cohorts of Norwegian large-duct PSC patients, one discovery panel (<jats:italic>n<\/jats:italic>\u2009=\u2009165) and one validation panel (<jats:italic>n<\/jats:italic>\u2009=\u200987). Serum activity of autotaxin was measured in diluted sera by a fluorometric enzymatic assay. Patients reaching an end-point, liver transplantation or death, (discovery panel: <jats:italic>n<\/jats:italic>\u2009=\u2009118 [71.5%]; validation panel: <jats:italic>n<\/jats:italic>\u2009=\u200935 [40.2%]), showed higher autotaxin activity compared with the other patients, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001 and <jats:italic>P<\/jats:italic>\u2009=\u20090.004, respectively. Kaplan-Meier survival analyses showed a strong association between increasing autotaxin activity and shorter liver transplant-free survival (discovery panel: <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001, validation panel: <jats:italic>P<\/jats:italic>\u2009=\u20090.001). There was no relationship between autotaxin activity and the presence of inflammatory bowel disease or occurrence of hepatobiliary malignancy. In a multivariable analysis, high autotaxin activity was associated with an increased risk of liver transplantation or death (hazard ratio 2.03 (95% confidence interval 1.21\u20133.40), <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.01), independent from Mayo risk score, an in-house enhanced liver fibrosis score and interleukin-8 in serum. In conclusion, increased serum autotaxin activity is associated with reduced liver transplant-free survival independent from Mayo risk score and markers of inflammation and fibrosis.<\/jats:p>","DOI":"10.1038\/s41598-019-44762-7","type":"journal-article","created":{"date-parts":[[2019,6,11]],"date-time":"2019-06-11T10:02:53Z","timestamp":1560247373000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Autotaxin activity predicts transplant-free survival in primary sclerosing cholangitis"],"prefix":"10.1038","volume":"9","author":[{"given":"Amandeep K.","family":"Dhillon","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9263-948X","authenticated-orcid":false,"given":"Andreas E.","family":"Kremer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9660-6290","authenticated-orcid":false,"given":"Martin","family":"Kummen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kirsten M.","family":"Boberg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1779-0833","authenticated-orcid":false,"given":"Ronald P. 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