Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan.

Aim: Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes, although recurrent PSC (rPSC) poses significant challenges. This study aimed to describe patient characteristics and identify potential risk factors of rPSC in pediatric LT recipients.

Methods: This retrospective study analyzed 13 pediatric patients who underwent LT for PSC at a single center. Patient characteristics, risk factors, and outcomes were compared between those with and without rPSC.

Results: The median age at PSC diagnosis was 5.2 years and at LT, 15.4 years. Inflammatory bowel disease (IBD) was present in 12 patients (92.3%), and four (30.7%) had overlapping autoimmune hepatitis (AIH) before LT. Two patients received grafts from living-related donors, and 11 from deceased donors. During a median follow-up of 53 months, 4 of the 13 patients (30.7%) developed rPSC at a median of 48.9 months post-LT. Patients with rPSC tend to be younger at PSC diagnosis. All rPSC cases were associated with IBD, and half had AIH overlap, though the frequency difference was not significant. Acute cellular rejection (ACR) was universal in rPSC patients (100%) compared to nonrecurrent cases (33.3%, p = 0.07). One case of rPSC developed pulmonary hypertension following rPSC and succumbed to PH crisis, resulting in a 5-year patient survival rate of 82%.

Conclusions: The recurrence rate was high in pediatric patients with PSC. The observed association with immune-activating conditions raises the possibility of utilizing immunologic interventions to prevent rPSC, although further prospective studies are warranted to clarify the underlying mechanisms.