# Primary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges

**Authors:** Sofia Svensson Di Giorgio, Chiara Maria Scandavini, Antonio Molinaro, Urban Arnelo, Roberto Valente

PMC · DOI: 10.3390/jcm15031149 · Journal of Clinical Medicine · 2026-02-02

## TL;DR

This paper reviews the diagnosis and management of primary sclerosing cholangitis, a rare liver disease that can lead to cirrhosis and cancer.

## Contribution

The paper provides a clinically oriented overview and highlights challenges in managing primary sclerosing cholangitis.

## Key findings

- PSC leads to progressive ductal obliteration and liver cirrhosis.
- The global prevalence of PSC is rising, reaching up to 31.7 cases per 100,000 individuals.
- Liver transplantation is often required due to severe complications like cholangiocarcinoma.

## Abstract

Primary sclerosing cholangitis is a rare, chronic, inflammatory disease of the biliary tree that leads to progressive ductal obliteration, hepatic dysfunction, and ultimately liver cirrhosis. Most patients eventually require liver transplantation or develop serious complications, the most severe being end-stage liver disease and cholangiocarcinoma. The global prevalence of PSC is rising and has been reported to reach up to 31.7 cases per 100,000 individuals, representing a significant challenge in both diagnosis and management. In this review, we aim to provide a clinically oriented overview of the diagnosis and management of PSC. Furthermore, we seek to highlight key challenges and areas of uncertainty that clinicians encounter in the diagnosis and treatment of patients with this complex disease.

## Linked entities

- **Diseases:** Primary sclerosing cholangitis (MONDO:0013433), cholangiocarcinoma (MONDO:0019087)

## Full-text entities

- **Diseases:** cholangiocarcinoma (MESH:D018281), hepatic dysfunction (MESH:D008107), inflammatory disease (MESH:D007249), end-stage liver disease (MESH:D058625), PSC (MESH:D015209), liver cirrhosis (MESH:D008103)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC12897808/full.md

## References

116 references — full list in the complete paper: https://tomesphere.com/paper/PMC12897808/full.md

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Source: https://tomesphere.com/paper/PMC12897808