ArticleHepatology (Baltimore, Md.)2017
Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis: An international cohort study.
Article in Hepatology (Baltimore, Md.), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 42 papers, 3 of them syntheses that pooled it.
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Who cites it
42 citing papers in PubMed, 3 syntheses or guidelines pooled it, 100 citations in OpenAlex.
- [Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2022Guideline
- Quality of life in primary sclerosing cholangitis: a systematic review.Health and quality of life outcomes · 2021Pooled it
- The role of imaging in determining prognosis for primary sclerosing cholangitis: A systematic review.Saudi journal of gastroenterology : official journal of the Saudi Gastroenterology AssociationPooled it
- Primary sclerosing cholangitis.Nature reviews. Disease primers · 2025Review
- Management of primary sclerosing cholangitis: Current state-of-the-art.Hepatology communications · 2024Review
- Primary sclerosing cholangitis: Is qualitative and quantitative 3 T MR imaging useful for the evaluation of disease severity?European journal of radiology open · 2024Article
- Protocol for the development of a core outcome set for clinical trials in primary sclerosing cholangitis.BMJ open · 2024Article
- Primary Sclerosing Cholangitis: Diagnostic Criteria.Tomography (Ann Arbor, Mich.) · 2024Review
- The Oral Cholate Challenge Test Quantifies Risk for Liver-Related Clinical Outcomes in Primary Sclerosing Cholangitis.Gastro hep advances · 2024Article
- Interobserver agreement and prognostic value of image-based scoring systems in patients with primary sclerosing cholangitis.Abdominal radiology (New York) · 2024Article
- Molecular magnetic resonance imaging of liver inflammation using an oxidatively activated probe.JHEP reports : innovation in hepatology · 2023Article
- A composite score using quantitative magnetic resonance cholangiopancreatography predicts clinical outcomes in primary sclerosing cholangitis.JHEP reports : innovation in hepatology · 2023Article
- Oxidative stress-related markers as prognostic factors for patients with primary sclerosing cholangitis in Japan.Hepatology international · 2023Article
- The Many Faces of Primary Sclerosing Cholangitis: Controversy Abounds.Digestive diseases and sciences · 2023Review
- Protective potential of the gallbladder in primary sclerosing cholangitis.JHEP reports : innovation in hepatology · 2023Article
- PRIMIS: design of a pivotal, randomized, phase 3 study evaluating the safety and efficacy of the nonsteroidal farnesoid X receptor agonist cilofexor in noncirrhotic patients with primary sclerosing cholangitis.BMC gastroenterology · 2023Article
- The microbiota and the gut-liver axis in primary sclerosing cholangitis.Nature reviews. Gastroenterology & hepatology · 2023Review
- Primary sclerosing cholangitis: review for radiologists.Abdominal radiology (New York) · 2023Review
- Primary sclerosing cholangitis-A long night's journey into day.Clinical liver disease · 2022Review
- Role of ductular reaction and ductular-canalicular junctions in identifying severe primary biliary cholangitis.JHEP reports : innovation in hepatology · 2022Article
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Authors and funding
27 authors at 17 institutions in 9 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 (PSC-related 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 (κ = 0.56) and substantial for Nakanuma component fibrosis (κ = 0.67), Ishak stage (κ = 0.64), and Ludwig stage (κ = 0.62).
conclusionWe 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. (Hepatology 2017;65:907-919).
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