Validation of the Prognostic Value of Histologic Scoring Systems in Primary Sclerosing Cholangitis: An International Cohort Study

被引：77

作者：

de Vries, Elisabeth M. G. ^{[1
]}

de Krijger, Manon ^{[1
]}

Faerkkilae, Martti ^{[2
,3
]}

Arola, Johanna ^{[4
,5
]}

Schirmacher, Peter ^{[6
]}

Gotthardt, Daniel ^{[6
,7
]}

Goeppert, Benjamin

Trivedi, Palak J. ^{[8
]}

Hirschfield, Gideon M. ^{[8
]}

Ytting, Henriette ^{[9
]}

Ben Vainer ^{[10
]}

van Buuren, Henk R. ^{[11
]}

Biermann, Katharina ^{[12
]}

Harms, Maren H. ^{[11
]}

Chazouilleres, Olivier

Wendum, Dominique ^{[14
]}

Kemgang, Astrid D. ^{[13
]}

Chapman, Roger W. ^{[15
,16
]}

Wang, Lai Mun ^{[17
,18
]}

Williamson, Kate D. ^{[15
,16
]}

Gouw, Annette S. H. ^{[19
]}

Paradis, Valerie ^{[20
]}

Sempoux, Christine ^{[21
]}

Beuers, Ulrich ^{[1
]}

Huebscher, Stefan G. ^{[8
,22
]}

Verheij, Joanne ^{[23
]}

Ponsioen, Cyriel Y. ^{[1
]}

机构：

[1] Acad Med Ctr, Dept Gastroenterol & Hepatol, Meibergdreef 9, NL-1105 AZ Amsterdam, Netherlands

[2] Univ Helsinki, Helsinki, Finland

[3] Helsinki Univ Hosp, Dept Gastroenterol, Helsinki, Finland

[4] Univ Helsinki, Dept Pathol, Helsinki, Finland

[5] Univ Helsinki Hosp, Helsinki, Finland

[6] Univ Heidelberg Hosp, Inst Pathol, Heidelberg, Germany

[7] Univ Heidelberg Hosp, Dept Gastroenterol & Hepatol, Heidelberg, Germany

[8] Univ Birmingham, Natl Inst Hlth Res, Inst Immunol & Immunotherapy, Birmingham Liver Biomed Res Unit, Birmingham, W Midlands, England

[9] Univ Copenhagen, Dept Hepatol, Dept Rigshospitalet, Copenhagen, Denmark

[10] Univ Copenhagen, Dept Pathol, Dept Rigshospitalet, Copenhagen, Denmark

[11] Erasmus Univ, Ctr Med, Dept Gastroenterol & Hepatol, Rotterdam, Netherlands

[12] Erasmus Univ, Ctr Med, Dept Pathol, Rotterdam, Netherlands

[13] Univ Paris 06, Sorbonne Univ, Hop Saint Antoine, AP HP,Dept Hepatol, Paris, France

[14] Univ Paris 06, Sorbonne Univ, Hop Saint Antoine, AP HP,Dept Pathol, Paris, France

[15] Univ Oxford, Nuffield Dept Med, Oxford, England

[16] John Radcliffe Hosp, Translat Gastroenterol Unit, Oxford, England

[17] Oxford Univ Hosp NHS Fdn Trust, John Radcliffe Hosp, Dept Cellular Pathol, Oxford, England

[18] Ludwig Inst Canc Res, Oxford, England

[19] Univ Groningen, Univ Med Ctr Groningen, Dept Pathol & Med Biol, Groningen, Netherlands

[20] Beaujon Hosp, Assistance Publ Hop Paris, Dept Pathol, Paris, France

[21] CHU Vaudois, IUP, Lausanne, Switzerland

[22] Queen Elizabeth Hosp, Dept Cellular Pathol, Birmingham, W Midlands, England

[23] Acad Med Ctr, Dept Pathol, Amsterdam, Netherlands

来源：

HEPATOLOGY | 2017年 / 65卷 / 03期

基金：

英国惠康基金;

关键词：

PRIMARY BILIARY-CIRRHOSIS; LIVER FIBROSIS; RISK-STRATIFICATION; STAGING SYSTEMS; GRADING SYSTEM; BIOPSY; INTEROBSERVER; INFLAMMATION;

D O I：

10.1002/hep.28963

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

100201 [内科学];

摘要：

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 PSC cohort. The aim of this study was to validate their prognostic use and reproducibility across a multicenter PSC cohort. Liver biopsies from PSC patients were collected from seven European institutions. 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 endpoints 1 (PSCrelated death and liver transplantation), 2 (liver transplantation), and 3 (liver-related events), was assessed using univariable and multivariable Cox proportional hazards modeling. A total of 119 PSC patients were identified, and the median follow-up was 142 months. During follow-up, 31 patients died (20 PSC-related deaths), 31 patients underwent liver transplantation, and 35 patients experienced one or more liver-related events. All three staging systems were independent predictors of endpoints 2 and 3 (Nakanuma system: hazard ratio [HR], 3.16 [95% confidence interval (CI), 1.49-6.68] for endpoint 2 and HR, 2.05 [95% CI, 1.17-3.57] for endpoint 3; Ishak system: HR, 1.55 [95% CI, 1.10-2.18] for endpoint 2 and HR, 1.43 [95% CI, 1.10-1.85] for endpoint 3; Ludwig system: HR, 2.62 [95% CI, 1.19-5.80] for endpoint 2 and HR, 2.06 [95% CI, 1.09-3.89] for endpoint 3). Only the Nakanuma staging system was independently associated with endpoint 1: HR, 2.14 (95% CI, 1.22-3.77). Interobserver agreement was moderate for Nakanuma stage (k = 0.56) and substantial for Nakanuma component fibrosis (k = 0.67), Ishak stage (k = 0.64), and Ludwig stage (k = 0.62). Conclusion: We confirm the independent prognostic value and demonstrate for the first time the reproducibility of staging disease progression in PSC using the Nakanuma, Ishak, and Ludwig staging systems. The Nakanuma staging system-incorporating features of chronic biliary disease-again showed the strongest predictive value.

引用

页码：907 / 919

页数：13

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