Prognosis and transplant-free survival in PSC

What is known

• In a Greek national cohort, cumulative transplant-free survival was 91.4% at 5 years and 85.4% at 10 years, and was higher in PSC-IBD than in PSC-only patients. ¹
• PSC accounts for up to 17% of all liver transplants performed globally; the absence of disease-modifying therapy necessitates timely transplantation in select cases. ²
• In childhood-onset PSC, transplant-free survival was 91% at 10 years, indicating a good prognosis during the first decade after diagnosis. ³

What is unknown / caveats

• Reported ranges are wide and cohort-dependent (pediatric, PSC-IBD vs PSC-only, national registries)
• Conventional prognostic tools such as MELD perform suboptimally in PSC
• The wide 9-21 year range reflects the clinical heterogeneity of PSC, so an individual prognosis varies considerably.

References

1. Theodoros V, Fotios F, Konstantinos M, Euaggelos C, Konstantinos A, George M, Konsantinos T, Paraskevi F, Spyridon M, Nikoletta M, Olga G, Evangelina V, George G, Ioannis D, Pantelis K, Maria T, Eirini Z, George T, Despoina A, Panagiota I, Margarita P, Christos L, Aggeliki C, Evanthia Z, Nikolaos K, Dimitrios N, Giorgos B, Jiannis V, Andreas K, Nikos V, Dimitrios C, George P. HERACLIS-PSC: Current characteristics of primary sclerosing cholangitis with or without inflammatory bowel disease in Greece-a national multicenter cohort study. Dig Liver Dis. 2026;58(3):338-345. PMID: 41577581.

2. Frolkis AD, Nayagam JS, Parente A, Seager M, Sadler MD, Montano-Loza AJ, Joshi D. Evaluation and management of primary sclerosing cholangitis patients awaiting liver transplantation. JHEP Rep. 2026. PMID: 41825099.

3. Jerregård Skarby A, Casswall T, Bergquist A, Lindström L. Good long-term outcomes of primary sclerosing cholangitis in childhood. JHEP Rep. 2024. PMID: 39139456.

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Draft, generated by the IBDology RAG and not yet clinician-reviewed. Answers are grounded in the retrieved literature listed above; a high faithfulness score means the answer matches its sources, not that the sources are correct.