Enhanced liver fibrosis test predicts transplant-free survival in primary sclerosing cholangitis, a multi-centre study
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Background & Aims: Biomarkers reflecting disease activity and prognosis in primary sclerosing cholangitis (PSC) have not been firmly established. Enhanced liver fibrosis (ELF) test was previously reported to predict outcome in PSC. We aimed to validate the prognostic utility of ELF test in an independent, multi‐centre, retrospective PSC study population. Methods: We collected serum samples from PSC patients from seven countries. We estimated rates of transplant‐free survival by the Kaplan‐Meier method, used Cox proportional hazards regression to explore the association between ELF test and clinical outcome and determined prognostic performance of ELF test by computing the area under the receiver operating characteristic (AUC‐ROC) curve. Results: The final analysis included 534 PSC patients (61% males). Features of autoimmune hepatitis or concomitant inflammatory bowel disease affected 44 (8%) and 379 (71%) patients respectively. ELF test levels were higher in patients reaching the combined endpoint liver transplantation or death (median 10.9 [Interquartile range (IQR): 9.8‐12.1]; n=24 deaths, 79 liver transplantations) compared to those censored (8.8 [IQR: 8.0‐9.8]); P<.001. ELF test expressed as mild, moderate and severe fibrosis was significantly associated with the risk of reaching the endpoint (P<.001). ELF test independently predicted clinical outcome (Hazard ratio 1.31; 95% confidence interval [1.05‐1.65]; P=.018), and enabled good discrimination between PSC patients with and without endpoint (AUC‐ROC: 0.79). Conclusion: Our retrospective data validates the predictive utility of ELF test for clinical outcomes in PSC. The clinical utility of biomarkers for fibrosis in patients with PSC should be assessed in prospective patient cohorts.
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The ELF test can predict transplant-free survival in PSC and retains predictive value longitudinally despite dynamic fluctuations in the course of PSC with regard to progression to cirrhosis, but without association between reduced values and improved outcome.
“The ELF test can predict transplant-free survival in PSC 81,82 and retains predictive value longitudinally despite dynamic fluctuations in the course of PSC with regard to progression to cirrhosis (but without association between reduced values and improved outcome).79”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [82]
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Jaeger, M.-C., Guiraud, L., Rolland, C., Rolland, C., Belot, A., Sebbag, M., Bermúdez‐Humarán, L. G., Langella, P., Vergnolle, N., & Deraison, C. (2019) · United European Gastroenterology Journal · doi:10.1177/2050640619854671 · Cited by 11 · see citing works
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Autotaxin activity predicts transplant-free survival in primary sclerosing cholangitis
Dhillon, A. K., Kremer, A. E., Kummen, M., Boberg, K. M., Elferink, R. P. J. O., Karlsen, T. H., Beuers, U. H., Vesterhus, M. N., & Hov, J. R. (2019) · Scientific Reports · doi:10.1038/s41598-019-44762-7 · Cited by 8 · see citing works
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Self-citationNGM282, an FGF19 analogue, in primary sclerosing cholangitis: A nebulous matter
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- Authors
- de Vries, E. M. G., Färkkilä, M., Milkiewicz, P., Hov, J. R., Eksteen, B., Thorburn, D., Chazouillères, O., Pares, A., Nygård, S., Gilja, O. H., Wunsch, E., Invernizzi, P., Carbone, M., Bernuzzi, F., Boberg, K. M., Røsjø, H., Rosenberg, W., Beuers, U. H., Ponsioen, C. Y., Karlsen, T. H., & Vesterhus, M.
- Affiliations
- Department of Gastroenterology and Hepatology Academic Medical Center Amsterdam The Netherlands
- University of Helsinki (FI)
- Medical University of Warsaw (PL)
- Oslo University Hospital (NO)
- University of Calgary (CA)
- Royal Free London NHS Foundation Trust (GB)
- Hôpital Saint-Antoine (FR)
- Liver Unit, Hospital Clínic, IDIBAPS, CIBERehd University of Barcelona Barcelona Spain
- Haukeland University Hospital (NO)
- Pomeranian Medical University (PL)
- Humanitas University (IT)
- University of Oslo (NO)
- Amsterdam UMC Location University of Amsterdam (NL)
- Corresponding author
- Vesterhus, Mette
- ORCID
- Färkkilä, Martti
- Milkiewicz, Piotr
- Hov, Johannes R.
- Eksteen, Bertus
- Thorburn, Douglas
- Chazouillères, Olivier
- Nygård, Ståle
- Gilja, Odd H.
- Wunsch, Ewa
- Invernizzi, Pietro
- Carbone, Marco
- Bernuzzi, Francesca
- Boberg, Kirsten M.
- Røsjø, Helge
- Rosenberg, William
- Beuers, Ulrich H.
- Ponsioen, Cyriel Y.
- Karlsen, Tom H.
- Vesterhus, Mette
- Venue
- Liver International
- Publisher
- Wiley
- Published
- 2017-04-13
- Volume
- 37
- Issue
- 10
- Pages
- 1554-1561
- ISSN
- 0106-9543 (ISSN-L)
- 1478-3223
- 1478-3231
- OpenAlex
- W2594051447
- Cited by
- 83
- References
- 26
- Citations by year
- 2026: 10 · 2025: 7 · 2024: 7 · 2023: 6 · 2022: 7 · 2021: 11 · 2020: 9 · 2019: 12 · 2018: 10 · 2017: 4
- FWCI
- 5.90
- Type
- journal article
- Language
- en
- Open access
- green
- pure.amsterdamumc.nl
- Licence
- Locations
- Liver International (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (acceptedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- BOA (University of Milano-Bicocca) (submittedVersion)
- NORA - Norwegian Open Research Archives (submittedVersion, other-oa)
- Dipòsit Digital de la Universitat de Barcelona (Universitat de Barcelona) (submittedVersion, public-domain)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Norwegian PSC Research Center
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Epidemiology › Liver Disease Diagnosis and Treatment
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Keywords
- primary sclerosing cholangitis, enhanced liver fibrosis test, liver fibrosis, transplant-free survival, prognostic factors, liver transplantation, disease activity, inflammatory bowel disease, autoimmune hepatitis, noninvasive fibrosis assessment
- MeSH terms
- Adult, Canada, Europe, Female, Humans, Hyaluronic Acid, Liver Cirrhosis, Male, Middle Aged, Peptide Fragments, Phenotype, Predictive Value of Tests, Procollagen, Retrospective Studies, Risk Factors, ROC Curve, Time Factors, Reproducibility of Results, Cholangitis, Sclerosing, Biomarkers, Multivariate Analysis, Proportional Hazards Models, Liver Transplantation, Disease Progression, Disease-Free Survival, Area Under Curve, Tissue Inhibitor of Metalloproteinase-1, Kaplan-Meier Estimate, Young Adult
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