# Evaluation and management of primary sclerosing cholangitis patients awaiting liver transplantation

## TL;DR

This review discusses the challenges and strategies for managing primary sclerosing cholangitis patients before liver transplantation, emphasizing the need for individualized care and early specialist referral.

## Contribution

The paper provides a comprehensive review of prognostic models and transplant decision-making in primary sclerosing cholangitis.

## Key findings

- Liver transplantation is the only definitive treatment for PSC, with over 80% survival at 5 years post-transplant.
- PSC recurrence occurs in about 25% of patients after liver transplantation.
- PSC-specific prognostic scores offer better risk stratification than conventional tools like the MELD score.

## Abstract

Primary sclerosing cholangitis (PSC) is a rare, progressive cholestatic liver disease characterised by biliary strictures, increased risk of cholangiocarcinoma, and a strong association with inflammatory bowel disease. Despite its low prevalence, PSC accounts for up to 17% of all liver transplants (LT) performed globally. Transplant-free survival ranges from a median of 9 to 21 years from diagnosis. LT is the only definitive treatment, with post-LT survival in PSC exceeding 80% at 5 years. However, PSC recurs in approximately 25% of individuals. The absence of disease-modifying therapy necessitates timely LT in select cases of hepatic decompensation, refractory pruritus, recurrent cholangitis, or selected early-stage malignancies. However, PSC poses unique challenges in pre-transplant assessment and timing due to its clinical heterogeneity and the suboptimal performance of conventional prognostic tools, such as the MELD score. Although PSC-specific scores offer improved risk stratification, they are not integrated into routine LT algorithms. This review synthesises the current landscape of prognostic modelling in PSC, focusing on validated tools and their role in transplant decision making. We examine the range of LT indications in PSC and explore the interplay between PSC and inflammatory bowel disease, with particular focus on malignancy surveillance and immunosuppression planning. We review the transplant evaluation process, including hepatobiliary imaging, endoscopy, and multidisciplinary approaches to high-grade stricture evaluation and oncologic risk assessment. By integrating contemporary evidence and expert opinion, this review aims to guide clinicians in the nuanced assessment and management of patients with PSC in the peri-transplant period, emphasising the need for individualised risk assessment and early referral to transplantation centres with hepatobiliary surgery and transplant expertise.

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