What prognostic tests are recommended for patients with Primary Sclerosing Cholangitis (PSC)?

From the Guidelines

The most recent and highest quality study recommends that patients with Primary Sclerosing Cholangitis (PSC) should undergo regular liver function tests, elastography techniques like FibroScan, MRCP, and the Enhanced Liver Fibrosis (ELF) test to assess disease severity and predict outcomes. According to the study published in the Journal of Hepatology in 2022 1, several prognostic tests are recommended for patients with PSC. The Mayo Risk Score is the most widely used prognostic model, incorporating age, bilirubin, albumin, AST, and history of variceal bleeding to predict survival.

Some key points to consider:

• Regular liver function tests (ALT, AST, ALP, GGT, bilirubin) should be performed every 3-6 months to monitor disease progression.
• Elastography techniques like FibroScan help assess liver fibrosis non-invasively and should be done annually.
• MRCP (Magnetic Resonance Cholangiopancreatography) is recommended every 1-2 years to evaluate bile duct changes and detect early cholangiocarcinoma.
• The Enhanced Liver Fibrosis (ELF) test measures serum biomarkers of fibrosis and provides prognostic information.
• Annual surveillance colonoscopy is crucial as PSC patients have increased risk of colorectal cancer.
• Regular ultrasound with CA 19-9 tumor marker testing helps monitor for cholangiocarcinoma development.

These tests collectively help clinicians stratify risk, guide treatment decisions, determine transplant timing, and detect complications early, improving patient management and outcomes in this progressive disease. Additionally, the study published in the Journal of Hepatology in 2021 1 suggests that the ELF score and liver stiffness measurement (LSM) by transient elastography (TE) correlate with outcomes and should be used for risk stratification both at baseline and during follow-up.

It's also important to note that the European Association for the Study of the Liver (EASL) clinical practice guidelines on sclerosing cholangitis recommend that cholangiocarcinoma (CCA) should be suspected in any patient with worsening cholestasis, weight loss, raised serum CA19-9, and/or new or progressive dominant stricture, particularly with an associated enhancing mass lesion 1.

Overall, a comprehensive approach to prognostic testing in PSC patients is essential to improve patient outcomes.

From the Research

Prognostic Tests for Primary Sclerosing Cholangitis (PSC)

The following prognostic tests are recommended for patients with PSC:

• Revised Mayo Risk Score (rMRS) 2, 3
• UK-PSC score 2
• PREsTo 2
• Enhanced liver fibrosis score 2
• Amsterdam-Oxford model 2, 4
• Time-dependent Cox regression model 5
• Short-term prognostic factors such as alkaline phosphatase (ALP) levels, albumin, bilirubin, international normalized ratio of prothrombin time (PT-INR), and Model for End-Stage Liver Disease (MELD) score 6

Key Findings

• The rMRS was better than the Amsterdam-Oxford model for predicting 1-year transplant-free survival 2
• The UK-PSC score outperformed the rMRS for 10-year transplant-free survival 2
• An enhanced liver fibrosis score was independently associated with transplant-free survival after adjusting for rMRS 2
• PREsTo predicts 5-year hepatic decompensation with a c-statistic modestly higher than rMRS 2
• ALP levels below 1.5 upper limit of normal (ULN) predicted better prognosis in PSC 6
• A time-dependent Cox regression model was superior to the time-fixed variant in assigning low 1-year survival probabilities to patients that actually survived less than 1 year 5

Prognostic Models

• The rMRS is a widely used prognostic model for PSC, which includes variables such as age, bilirubin, aspartate aminotransferase, and albumin 3
• The UK-PSC score is a newer prognostic model that has been shown to be more accurate than the rMRS in predicting clinical endpoints in PSC 2
• The PREsTo model is a prognostic model that predicts 5-year hepatic decompensation in PSC patients 2
• The Amsterdam-Oxford model is a prognostic model that has been shown to be adequate in estimating PSC-related death and/or liver transplant in a predominantly population-based setting 4