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Enhanced liver fibrosis score predicts transplant-free survival in primary sclerosing cholangitis
Open accessAbstract
There is a need to determine biomarkers reflecting disease activity and prognosis in primary sclerosing cholangitis (PSC). We evaluated the prognostic utility of the enhanced liver fibrosis (ELF) score in Norwegian PSC patients. Serum samples were available from 305 well‐characterized large‐duct PSC patients, 96 ulcerative colitis patients, and 100 healthy controls. The PSC patients constituted a derivation panel (recruited 1992‐2006 [n = 167]; median age 41 years, 74% male) and a validation panel (recruited 2008‐2012 [n = 138]; median age 40 years, 78% male). We used commercial kits to analyze serum levels of hyaluronic acid, tissue inhibitor of metalloproteinases‐1, and propeptide of type III procollagen and calculated ELF scores by the previously published algorithm. Results were also validated by analysis of ELF tests using the ADVIA Centaur XP system and its commercially available reagents. We found that PSC patients stratified by ELF score tertiles exhibited significantly different transplant‐free survival in both panels ( P < 0.001), with higher scores associated with shorter survival, which was confirmed in the validation panel stratified by ELF test tertiles ( P = 0.003). The ELF test distinguished between mild and severe disease defined by clinical outcome (transplantation or death) with an area under the curve of 0.81 (95% confidence interval [CI] 0.73‐0.87) and optimal cutoff of 10.6 (sensitivity 70.2%, specificity 79.1%). In multivariate Cox regression analysis in both panels, ELF score (hazard ratio = 1.9, 95% CI 1.4‐2.5, and 1.5, 95% CI 1.1‐2.1, respectively) was associated with transplant‐free survival independently of the Mayo risk score (hazard ratio = 1.3, 95% CI 1.1‐1.6, and 1.6, 95% CI 1.2‐2.1, respectively). The ELF test correlated with ultrasound elastography in separate assessments. Conclusion : The ELF score is a potent prognostic marker in PSC, independent of the Mayo risk score. (H epatology 2015;62:188‐197)
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“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 [81]
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Self-citationSerum markers detect the presence of liver fibrosis: A cohort study
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Self-citationNatural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.
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Self-citationSolberg, I. C., Lygren, I., Jahnsen, J., Aadland, E., Høie, O., Cvancarova, M., Bernklev, T., Henriksen, M., Sauar, J., Vatn, M. H., et al. (2008) · Scandinavian Journal of Gastroenterology · doi:10.1080/00365520802600961 · Cited by 729
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Berzigotti, A., Tsochatzis, E., Boursier, J., Castera, L., Cazzagon, N., Friedrich-Rust, M., Petta, S., & Thiele, M. (2021) · Journal of Hepatology · doi:10.1016/j.jhep.2021.05.025 · Cited by 1,925
Primary sclerosing cholangitis – a comprehensive review
Karlsen, T. H., Folseraas, T., Thorburn, D., & Vesterhus, M. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.07.022 · Cited by 868
Self-citationGómez, E. V., & Chalasani, N. (2017) · Journal of Hepatology · doi:10.1016/j.jhep.2017.11.013 · Cited by 608
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Self-citationPrimary sclerosing cholangitis
Dyson, J. K., Beuers, U. H., Jones, D. E. J., Lohse, A. W., & Hudson, M. (2018) · The Lancet · doi:10.1016/s0140-6736(18)30300-3 · Cited by 496
Metadata
- Authors
- Vesterhus, M., Hov, J. R., Holm, A., Schrumpf, E., Nygård, S., Godang, K., Andersen, I. M., Næss, S., Thorburn, D., Saffioti, F., Vatn, M., Gilja, O. H., Lund‐Johansen, F., Syversveen, T., Brabrand, K., Parés, A., Ponsioen, C. Y., Pinzani, M., Färkkilä, M., Moum, B., Ueland, T., Røsjø, H., Rosenberg, W., Boberg, K. M., & Karlsen, T. H.
- Affiliations
- Oslo University Hospital (NO)
- Royal Free London NHS Foundation Trust (GB)
- Corresponding author
- Karlsen, Tom H.
- ORCID
- Vesterhus, Mette
- Hov, Johannes Roksund
- Nygård, Ståle
- Godang, Kristin
- Andersen, Ina Marie
- Thorburn, Douglas
- Saffioti, Francesca
- Gilja, Odd Helge
- Lund‐Johansen, Fridtjof
- Syversveen, Trygve
- Parés, Albert
- Ponsioen, Cyriel Y.
- Pinzani, Massimo
- Färkkilä, Martti
- Moum, Bjørn
- Ueland, Thor
- Røsjø, Helge
- Rosenberg, William
- Boberg, Kirsten Muri
- Karlsen, Tom H.
- Venue
- Hepatology
- Published
- 2015-04-28
- Volume
- 62
- Issue
- 1
- Pages
- 188-197
- ISSN
- 0270-9139 (ISSN-L)
- 1527-3350
- OpenAlex
- W1869119189
- Cited by
- 138
- References
- 41
- Citations by year
- 2026: 10 · 2025: 7 · 2024: 11 · 2023: 12 · 2022: 10 · 2021: 13 · 2020: 12 · 2019: 16 · 2018: 16 · 2017: 16 · 2016: 10 · 2015: 5
- FWCI
- 9.27
- Type
- journal article
- Language
- en
- Open access
- green
- pure.amsterdamumc.nl
- Licence
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (submittedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- National Institute for Health and Care Research (NF-SI-0512-10124)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Hepatology › Liver Disease Diagnosis and Treatment
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Keywords
- primary sclerosing cholangitis, enhanced liver fibrosis test, transplant-free survival, prognostic factors, liver fibrosis, hyaluronic acid, TIMP-1, Mayo risk score, ultrasound elastography, disease activity, ulcerative colitis, liver transplantation, serum biomarkers
- MeSH terms
- Adult, Female, Fibrosis, Humans, Liver, Male, Middle Aged, Norway, Severity of Illness Index, Cholangitis, Sclerosing, Case-Control Studies
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