Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis

Show full item record



Sjöblom , N , Boyd , S , Kautiainen , H , Arola , J & Färkkilä , M 2022 , ' Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis ' , Histopathology , vol. 81 , pp. 192-204 .

Title: Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis
Author: Sjöblom, Nelli; Boyd, Sonja; Kautiainen, Hannu; Arola, Johanna; Färkkilä, Martti
Contributor organization: HUSLAB
Department of Pathology
University of Helsinki
Department of General Practice and Primary Health Care
Helsinki University Hospital Area
Centre of Excellence in Complex Disease Genetics
HUS Abdominal Center
Department of Medicine
Gastroenterologian yksikkö
Date: 2022-08
Language: eng
Number of pages: 13
Belongs to series: Histopathology
ISSN: 0309-0167
Abstract: Background Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease that may lead to liver cirrhosis or cholangiocarcinoma. Liver histology and fibrosis stage are predictive markers of disease progression, and histological cirrhosis is defined as a significant endpoint. PSC-specific histological scoring methods are lacking at present. We aimed to develop a tailored classification system for PSC, the PSC histoscore, based on histological features associated with disease progression. Methods In total, 300 PSC patients diagnosed between 1988 and 2018 were enrolled; their data were collected from the PSC registry (Helsinki University Hospital), and liver specimens were obtained from the Biobank of Helsinki. Five histological features included in the adapted Nakanuma scoring system and three additional parameters typical for PSC histology were evaluated and compared with the clinical and laboratory data. A compound endpoint consisting of liver transplantation, development of cholangiocarcinoma, or death was used as outcome measurement. Results Stage (fibrosis, bile duct loss, ductular reaction, and chronic cholestasis) and grade (portal inflammation, portal edema, hepatitis activity, and cholangitis activity) parameters were found to be independent predictive risk factors for the compound endpoint (P < 0.001). High disease grade (2-6) and stage (2-4) better correlated with clinical endpoints when evaluated with the PSC histoscore system compared to the adapted Nakanuma classification. The risk for disease progression in sequential endoscopic retrograde cholangiography (ERC) examinations was increased with elevated total PSC histoscores. Conclusion The PSC histoscore is a novel histological classification system for PSC. Our findings support the applicability of liver histology as a marker for disease progression.
Subject: cholestatic liver disease
liver histology
prognostic tools
Nakanuma classification
PSC histoscore
3111 Biomedicine
1182 Biochemistry, cell and molecular biology
Peer reviewed: Yes
Usage restriction: openAccess
Self-archived version: publishedVersion

Files in this item

Total number of downloads: Loading...

Files Size Format View
Histopathology_ ... _in_primary_sclerosing.pdf 1.417Mb PDF View/Open

This item appears in the following Collection(s)

Show full item record