Post-Transplant Disease Recurrence in Pediatric PSC

Review the current knowledge about recurrent primary sclerosing cholangitis (rPSC) after transplant in children.

Recurrent PSC is a significant complication that afflicts 16% of children after liver transplantation for primary sclerosing cholangitis (PSC) at a median onset of 38 months post-transplant. Possible risk factors include younger age at PSC diagnosis or transplant, the presence of overlap syndrome or IBD, and post-transplant induction with thymoglobulin. rPSC impairs the patient's quality of life and can be detrimental to the graft. Preventive options and therapeutic measures are limited. Ursodeoxycholic acid is widely used, but its effect on long-term outcome is unknown. Vancomycin can improve the biochemical profile of rPSC, but it remains unknown whether it halts the disease progression. Pediatric liver transplant for primary sclerosis cholangitis can be complicated by recurrence of the disease, which portends poor outcomes. Although few risk factors have been implicated, larger studies with longer follow-ups are needed to characterize cardinal risk factors for rPSC, as well as evaluate possible preventative and therapeutic options.