Systematic Review

Non-invasive Prognostic Tests for Primary Sclerosing Cholangitis

Areej Mazhar; Mark W. Russo

Disclosures

Aliment Pharmacol Ther. 2021;53(7):774-783. 

In This Article

Abstract and Introduction

Abstract

Background: Several prognostic tests for primary sclerosing cholangitis (PSC) have been developed, including biochemical models, elastography and magnetic resonance imaging scores.

Aim: To conduct a systematic review of non-invasive prognostic tests for PSC.

Methods: A systematic review was conducted from 1987 to 2020 of blood tests, biochemical models, elastography and imaging scores associated with outcomes in PSC.

Results: Forty 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.

Conclusion:MRS 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.

Introduction

Primary sclerosing cholangitis (PSC) is characterised by diffuse inflammation and fibrosis of the biliary tree resulting in biliary cirrhosis and eventually hepatic failure. PSC is diagnosed by cholestatic liver serum biochemistry as well as imaging findings on MRCP or ERCP. A liver biopsy is not necessary for the diagnosis unless small duct PSC or an overlap syndrome are being considered. Speculated mechanisms of disease include autoimmunity, effects of cytokines and abnormalities in bile acid transporters resulting in liver injury.[1]

PSC is associated with a median survival of 21.3 years after diagnosis and up to 10% of patients develop cholangiocarcinoma.[2] There is no effective medical therapy for PSC. Liver transplantation is curative in patients with end stage liver disease from PSC, but the disease recurs in up to 20% of recipients.[3] Given the lack of effective therapy for PSC, there has been great interest in drug development. Thus, important surrogate endpoints for clinical trials are needed.

Important clinical outcomes include the development of cirrhosis, liver related complications, transplant-free survival and cholangiocarcinoma, but these outcomes may take years to occur. Thus, prognostic models are needed that predict clinical outcomes. Several biomarkers have been studied as predictors of outcomes in patients with PSC. Perhaps the most studied marker is alkaline phosphatase, but it has limited predictive value. Novel biochemical scores, transient elastography and MRI with MRCP have recently been evaluated to predict outcomes in PSC. The aim of this study was to conduct a systematic review of non-invasive prognostic tests for PSC.

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