Liver transplant in primary sclerosing cholangitis: Current trends and future directions

Abstract

Primary sclerosing cholangitis (PSC) is a chronic and progressive immune-mediated cholangiopathy causing biliary tree inflammation and scarring, leading to liver cirrhosis and end-stage liver disease. Diagnosis of PSC is challenging due to its nonspecific symptoms and overlap with other liver diseases. Despite the rising incidence of PSC, there is no proven medical therapy that can alter the natural history of the disease. While liver transplantation (LT) is the most effective approach for managing advanced liver disease caused by PSC, post-transplantation recurrence of PSC remains a challenge. Therefore, ongoing research aims to develop better therapies for PSC, and continued efforts are necessary to improve outcomes for patients with PSC. This article provides an overview of PSC's pathogenesis, clinical presentation, and management options, including LT trends and future aspects. It also highlights the need for improved therapeutic options and ethical considerations in providing equitable access to LT for patients with PSC. Additionally, the impact of liver transplant on the quality of life and psychological outcomes of patients with PSC is discussed. Ongoing research into PSC's pathogenesis and post-transplant recurrence is crucial for improved understanding of the disease and more effective treatment options.

Keywords: Liver transplantation; Management; Pathogenesis; Primary sclerosing cholangitis; Psychosocial outcomes.

Figure 1

Pathogenesis of disease progression in primary sclerosing cholangitis.

Figure 2

A cholangiography showing common findings in primary sclerosing cholangitis i.e the presence of multiple short narrowings (shown by arrows) and dilatations in the intra and extrahepatic bile ducts, creating a distinctive "beaded pattern".