In pediatric patients with PSC, does the presence or absence of IBD affect long-term prognosis regarding liver transplantation

What is known

- A pediatric PSC cohort with 93% IBD showed good prognosis in the first decade, with an unfavorable outcome tied to a high SCOPE (severity) index at diagnosis rather than IBD status. ¹ - An increased need for liver transplantation was reported in PSC-AIH overlap versus PSC alone, though based on few events and possibly confounded by higher fibrosis stage. ¹ - In a long-term pediatric PSC study, AIH overlap did not affect survival; shorter transplant-free survival was associated with lower platelet count, splenomegaly, and older age. ²

What is unknown / caveats

- Retrieved studies on IBD and PSC transplant outcomes are largely adult cohorts or focus on IBD course after transplant, not pre-transplant prognosis in children - No retrieved pediatric analysis directly compares transplant-free survival in PSC with versus without IBD - None of the retrieved pediatric excerpts isolates IBD presence/absence as a predictor of transplant-free survival, so its independent prognostic effect in children cannot be determined here.

## References

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

2. Feldstein AE, Perrault J, El-Youssif M, Lindor KD, Freese DK, Angulo P. Primary sclerosing cholangitis in children: a long-term follow-up study. Hepatology. 2003. PMID: 12830004.

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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._