Systematic review: non‐invasive prognostic tests for primary sclerosing cholangitis

Abstract

SummaryBackgroundSeveral prognostic tests for primary sclerosing cholangitis (PSC) have been developed, including biochemical models, elastography and magnetic resonance imaging scores.AimTo conduct a systematic review of non‐invasive prognostic tests for PSC.MethodsA systematic review was conducted from 1987 to 2020 of blood tests, biochemical models, elastography and imaging scores associated with outcomes in PSC.ResultsForty studies of prognostic tests that collectively included 16 094 subjects with PSC were reviewed, of which 26 studies of non‐invasive tests including 13 759 subjects with PSC were included. Normalisation or reduction of alkaline phosphatase with or without therapy was associated with transplant‐free survival and reduced risk of hepatobiliary cancers but cut‐off values for alkaline phosphatase were not consistent among studies. The most studied prognostic biochemical model was the Mayo Risk Score (MRS) evaluated in 18 studies with a c‐statistic from 0.63 to 0.85 for clinical outcomes. One study demonstrated that the UK‐PSC score outperforms MRS for predicting clinical outcomes with a c‐statistics of 0.81and 0.75 respectively. A transient elastography score greater than 11.1 kPa is associated with survival and liver‐related complications. The Anali score, derived from specific MRI and MRCP features, is associated with the development of cholangiocarcinoma and decompensated cirrhosis. Promising prognostic models include the enhanced liver fibrosis (ELF) score, ELF test and PREsTo scores.ConclusionMRS is the most studied prognostic score for clinical outcomes in PSC but the UK‐PSC score and PREsTo have better test performance. Further studies comparing MRS to UK‐PSC score, PREsTo or ELF with elastography or imaging‐based scores are warranted.

Current Therapeutics in Primary Sclerosing Cholangitis

“…It demonstrated that normalization or reduction of ALP was associated with improved transplant-free survival and reduced risk of hepatobiliary cancers. 44 In a prospective observational study, this clinical benefit was only significant if ALP reduction was achieved below 1.5 times upper limit of normal, and not by 40% of baseline serum levels. 45 Serum ALP is currently accepted as a reasonable surrogate endpoint in clinical trials or to assess response to therapy in clinical practice, 8 , 9 but will likely benefit from combination with other exploratory parameters and with defined cut-off values.…”

“…It shown superiority to the Child Pugh scoring system in predicting short-term survival, but its application is fairly limited to cirrhotic patients and only allows for prediction up to 4 years. 44 Other novel scores that have outperformed the MRS in predicting survival include the UK-PSC score, Amsterdam-Oxford model and Primary sclerosing cholangitis Risk Estimate Tool, 44 which should be considered as secondary endpoints in future clinical trials.…”

Elevated Liver Fibrosis Progression in Isolated PSC Patients and Increased Malignancy Risk in a PSC-IBD Cohort: A Retrospective Study

“…Prognostic scores were evaluated to assess the clinical courses of PSC and are presented in Table 2. Using the Mayo Risk Score as a parameter of PSC disease progression [21], a significant higher overall score was found in patients with isolated PSC compared to patients with coexisting IBD (2.46 (1.22-3.56) vs. 1.05 (0-2.28), p = 0.015; Table 2). The Amsterdam-Oxford PSC score, Child-Pugh-Score and MELD Score, however, showed no significant differences between the two cohorts (Amsterdam-Oxford-PSC Score: 2.27 (3.11-1.99) vs. 2.16 (1.66-2.97), p = 0.401; Child-Pugh Score: 7 (5-8) vs. 7 (5-8), p = 0.256; Meld-Score: 13 (7-16) vs. 12 (7-16), p = 0.873; Table 2).…”

Documento de posicionamiento de la «Societat Catalana de Digestologia» sobre elastografía hepática 2022

“…[43] Por otro lado, en pacientes con CBP y CEP un umbral de 9,5-10 kPa ha demostrado ser útil para identificar fibrosis avanzada. [44] Sin embargo, las variables de confusión son frecuentes y el número de pacientes limitado en estos estudios. En los pacientes con consumo activo de alcohol la rigidez hepática puede estar sobreestimada por la inflamación por lo que puede ser necesario repetir la ET cuando se considere que la inflamación ha remitido (2-4 semanas de abstinencia o reducción del consumo con mejoría bioquímica).…”