Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.

<p>Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single cohort of PSC patients. The aim of this study was to validate their prognostic uti...

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Main Authors: de Vries, E, de Krijger, M, Färkkilä, M, Arola, J, Schirmacher, P, Gotthardt, D, Goeppert, B, Trivedi, P, Hirschfield, G, Ytting, H, Vainer, B, Buuren, H, Biermann, K, Harms, M, Chazouilleres, O, Wendum, D, Kemgang, A, Chapman, R, Wang, L, Williamson, K, Gouw, A, Paradis, V, Sempoux, C, Beuers, U, Hübscher, S, Verheij, J, Ponsioen, C
Format: Journal article
Language:English
Published: Wiley 2016
_version_ 1797052109953171456
author de Vries, E
de Krijger, M
Färkkilä, M
Arola, J
Schirmacher, P
Gotthardt, D
Goeppert, B
Trivedi, P
Hirschfield, G
Ytting, H
Vainer, B
Buuren, H
Biermann, K
Harms, M
Chazouilleres, O
Wendum, D
Kemgang, A
Chapman, R
Wang, L
Williamson, K
Gouw, A
Paradis, V
Sempoux, C
Beuers, U
Hübscher, S
Verheij, J
Ponsioen, C
author_facet de Vries, E
de Krijger, M
Färkkilä, M
Arola, J
Schirmacher, P
Gotthardt, D
Goeppert, B
Trivedi, P
Hirschfield, G
Ytting, H
Vainer, B
Buuren, H
Biermann, K
Harms, M
Chazouilleres, O
Wendum, D
Kemgang, A
Chapman, R
Wang, L
Williamson, K
Gouw, A
Paradis, V
Sempoux, C
Beuers, U
Hübscher, S
Verheij, J
Ponsioen, C
author_sort de Vries, E
collection OXFORD
description <p>Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single cohort of PSC patients. The aim of this study was to validate their prognostic utility and reproducibility, using a well-characterized multicenter PSC cohort.</p> <br/> <p>Liver biopsies from PSC patients were collected across 7 European centers. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems. Biopsies were independently scored by six liver pathologists for interobserver agreement. The prognostic value of clinical, biochemical, and all three histologic scoring systems on predicting composite endpoint 1: PSC-related death and liver transplantation, 2: liver transplantation, and 3: liver related events, were assessed using uni- and multivariable Cox proportional hazards modelling.</p> <br/> <p>119 PSC patients were identified, The median follow-up was 142 months. During follow-up, 31 patients died (20 PSC-related deaths), 31 underwent liver transplantation, and 35 experienced one or more liver-related events. All three staging systems were independent predictors of endpoints 2 and 3; Nakanuma HR 3.16 (95%CI 1.49-6.68), HR 2.05 (95%CI 1.17-3.57); Ishak: HR 1.55 (95%CI 1.10-2.18), HR 1.43 (95%CI 1.10-1.85); Ludwig: HR 2.62 (95%CI 1.19-5.80), HR 2.06 (95%CI 1.09-3.89), respectively. Only the Nakanuma staging system was independently associated with endpoint 1: HR 2.14 (95%CI 1.22-3.77). The inter-observer agreement was moderate for Nakanuma stage (κ=0.56), and substantial for Nakanuma component fibrosis (κ=0.67), Ishak stage (κ=0.64) and Ludwig stage (κ=0.62).</p> <h4>Conclusion</h4> <p>We confirm the independent prognostic value and reproducibility of predicting disease progression in PSC by the Nakanuma, Ishak and Ludwig staging systems. The Nakanuma staging system – that incorporates features of chronic biliary disease – showed to hold the strongest predictive value.</p>
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spelling oxford-uuid:08afcfff-57cf-4f56-b886-dac84a1523542022-03-26T09:14:18ZValidation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:08afcfff-57cf-4f56-b886-dac84a152354EnglishSymplectic Elements at OxfordWiley2016de Vries, Ede Krijger, MFärkkilä, MArola, JSchirmacher, PGotthardt, DGoeppert, BTrivedi, PHirschfield, GYtting, HVainer, BBuuren, HBiermann, KHarms, MChazouilleres, OWendum, DKemgang, AChapman, RWang, LWilliamson, KGouw, AParadis, VSempoux, CBeuers, UHübscher, SVerheij, JPonsioen, C <p>Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single cohort of PSC patients. The aim of this study was to validate their prognostic utility and reproducibility, using a well-characterized multicenter PSC cohort.</p> <br/> <p>Liver biopsies from PSC patients were collected across 7 European centers. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems. Biopsies were independently scored by six liver pathologists for interobserver agreement. The prognostic value of clinical, biochemical, and all three histologic scoring systems on predicting composite endpoint 1: PSC-related death and liver transplantation, 2: liver transplantation, and 3: liver related events, were assessed using uni- and multivariable Cox proportional hazards modelling.</p> <br/> <p>119 PSC patients were identified, The median follow-up was 142 months. During follow-up, 31 patients died (20 PSC-related deaths), 31 underwent liver transplantation, and 35 experienced one or more liver-related events. All three staging systems were independent predictors of endpoints 2 and 3; Nakanuma HR 3.16 (95%CI 1.49-6.68), HR 2.05 (95%CI 1.17-3.57); Ishak: HR 1.55 (95%CI 1.10-2.18), HR 1.43 (95%CI 1.10-1.85); Ludwig: HR 2.62 (95%CI 1.19-5.80), HR 2.06 (95%CI 1.09-3.89), respectively. Only the Nakanuma staging system was independently associated with endpoint 1: HR 2.14 (95%CI 1.22-3.77). The inter-observer agreement was moderate for Nakanuma stage (κ=0.56), and substantial for Nakanuma component fibrosis (κ=0.67), Ishak stage (κ=0.64) and Ludwig stage (κ=0.62).</p> <h4>Conclusion</h4> <p>We confirm the independent prognostic value and reproducibility of predicting disease progression in PSC by the Nakanuma, Ishak and Ludwig staging systems. The Nakanuma staging system – that incorporates features of chronic biliary disease – showed to hold the strongest predictive value.</p>
spellingShingle de Vries, E
de Krijger, M
Färkkilä, M
Arola, J
Schirmacher, P
Gotthardt, D
Goeppert, B
Trivedi, P
Hirschfield, G
Ytting, H
Vainer, B
Buuren, H
Biermann, K
Harms, M
Chazouilleres, O
Wendum, D
Kemgang, A
Chapman, R
Wang, L
Williamson, K
Gouw, A
Paradis, V
Sempoux, C
Beuers, U
Hübscher, S
Verheij, J
Ponsioen, C
Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title_full Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title_fullStr Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title_full_unstemmed Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title_short Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
title_sort validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis an international cohort study
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