ArticleHepatology (Baltimore, Md.)2021
The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.
Article in Hepatology (Baltimore, Md.), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 2 of them syntheses that pooled it.
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16 citing papers in PubMed, 2 syntheses or guidelines pooled it, 39 citations in OpenAlex.
- Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group.Journal of pediatric gastroenterology and nutrition · 2025Guideline
- [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
- The Childhood Liver Disease Research Network's prospective characterization of pediatric primary sclerosing cholangitis.Hepatology communications · 2026Observational
- Autoimmune liver diseases and overlap syndromes in children with inflammatory bowel diseases.European journal of pediatrics · 2026Review
- Primary sclerosing cholangitis in children: a single-center experience and evaluation of prognostic markers.Translational gastroenterology and hepatology · 2026Article
- Prognostic value of magnetic resonance imaging in pediatric-onset primary sclerosing cholangitis.Therapeutic advances in gastroenterology · 2026Article
- Primary sclerosing cholangitis.Nature reviews. Disease primers · 2025Review
- Primary sclerosing cholangitis: a narrative review of diagnostic and prognostic biomarkers.Translational gastroenterology and hepatology · 2025Review
- Management of primary sclerosing cholangitis: Current state-of-the-art.Hepatology communications · 2024Review
- Good long-term outcomes of primary sclerosing cholangitis in childhood.JHEP reports : innovation in hepatology · 2024Article
- TheEClinicalMedicine · 2024Article
- African American race does not confer an increased risk of clinical events in patients with primary sclerosing cholangitis.Hepatology communications · 2024Article
- Liver transplant in primary sclerosing cholangitis: Current trends and future directions.World journal of hepatology · 2023Review
- Natural history and prognosis of pediatric PSC with updates on management.Clinical liver disease · 2023Article
- Gamma-glutamyl transferase and disease course in pediatric-onset primary sclerosing cholangitis: A single-center cohort study.Health science reports · 2023Article
- Use of oral vancomycin in children with autoimmune liver disease: A single centre experience.World journal of hepatology · 2021Article
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57 authors at 20 institutions in 16 countries.
Funding
Abstract
BACKGROUND AND
aimsDisease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC. APPROACH AND
resultsWe used retrospective data from the Pediatric PSC Consortium. The training cohort contained 1,012 patients from 40 centers. We generated a multivariate risk index (Sclerosing Cholangitis Outcomes in Pediatrics [SCOPE] index) that contained total bilirubin, albumin, platelet count, gamma glutamyltransferase, and cholangiography to predict a primary outcome of liver transplantation or death (TD) and a broader secondary outcome that included portal hypertensive, biliary, and cancer complications termed hepatobiliary complications (HBCs). The model stratified patients as low, medium, or high risk based on progression to TD at rates of <1%, 3%, and 9% annually and to HBCs at rates of 2%, 6%, and 13% annually, respectively (P < 0.001). C-statistics to discriminate outcomes at 1 and 5 years were 0.95 and 0.82 for TD and 0.80 and 0.76 for HBCs, respectively. Baseline hepatic fibrosis stage was worse with increasing risk score, with extensive fibrosis in 8% of the lowest versus 100% with the highest risk index (P < 0.001). The model was validated in 240 children from 11 additional centers and performed well.
conclusionsThe SCOPE index is a pediatric-specific prognostic tool for PSC. It uses routinely obtained, objective data to predict a complicated clinical course. It correlates strongly with biopsy-proven liver fibrosis. SCOPE can be used with families for shared decision making on clinical care based on a patient's individual risk, and to account for variable disease progression when designing future clinical trials.
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