Sirolimus to treat chronic and steroid-resistant allograft rejection-related fibrosis in pediatric liver transplantation

• Published in: Journal of pediatric gastroenterology and nutrition
• Main Authors: Quintero Bernabeu, Jesús, Juamperez Goñi, Javier, Mercadal Hally, Maria, Padros Fornieles, Crsitina, Ortega López, Juan, Larrarte King, Mauricio, Molino Gahete, José A, Salcedo Allende, María T, Hidalgo Llompart, Ernest, Bilbao Aguirre, Itxarone, Charco Torra, Ramon
• Format: Journal Article
• Language: English
• Published: United States 01.11.2024
• Subjects: biliary injury; immunosuppression; mammalian target of rapamycin inhibitors
• ISSN: 1536-4801
• DOI: 10.1002/jpn3.12286

This study aimed to report our experience with the use of sirolimus in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with sirolimus for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after postsirolimus introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue sirolimus treatment owing to nephrotic proteinuria. In conclusion, sirolimus may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.