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Long-term results of pediatric liver transplantation for autoimmune liver disease
Eduardo Couchonnal1, Emmanuel Jacquemin2, Alain Lachaux3
1Hospices Civils de Lyon, Hôpital Femme-Mère-Enfant, Service d'Hépato-gastroentérologie et Nutrition Pédiatrique, Bron, France.
Insights
Long-term liver transplantation (LT) outcomes for pediatric autoimmune liver disease are concerning, with high rates of graft rejection and disease recurrence impacting survival. Early LT and fulminant liver failure predict poorer outcomes.
Area of Science:
- Pediatric Hepatology
- Transplantation Immunology
- Autoimmune Liver Diseases
Background:
- Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are rare causes of pediatric liver failure.
- Liver transplantation (LT) is a critical intervention for end-stage liver disease in children.
- Long-term outcomes following LT for these conditions require further investigation.
Purpose of the Study:
- To evaluate the long-term outcomes of pediatric patients undergoing liver transplantation for autoimmune liver diseases.
- To identify factors influencing survival and complications after LT for AIH and PSC.
- To assess the incidence of graft rejection and disease recurrence post-LT.
Main Methods:
- A retrospective multicenter study collected data from 30 children who received their first LT between 1988 and 2018.
- Patient demographics, indications for LT, complications, and survival rates were analyzed.
- Statistical analysis identified predictive factors for mortality.
Main Results:
- The study included 14 AIH type 1, 7 AIH type 2, and 9 PSC cases. Mean age at LT was 11.8 years.
- Graft rejection occurred in 63.3% of patients, with 20% experiencing disease recurrence (primarily AIH type 1).
- Significant predictors of death included age at LT < 1 year, LT for fulminant liver failure, and LT for AIH type 2.
Conclusions:
- Long-term outcomes for pediatric autoimmune liver disease patients undergoing LT are often compromised.
- Graft rejection and disease recurrence are significant complications, particularly in AIH.
- Age at transplantation and indication for LT are crucial factors affecting long-term survival.
Background:
Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are rare indications for liver transplantation (LT) in children. The aim of the present retrospective multicenter study was to evaluate long-term outcome after LT for autoimmune liver disease in childhood.
Methods:
Retrospective data from 30 children who underwent a first LT from 1988 to 2018 were collected.
Results:
The study population consisted of 18 girls and 12 boys, transplanted for AIH type 1 (n=14), AIH type 2 (n=7) or PSC (n=9). Mean age at LT was 11.8±5.2 years. The main indications for LT were acute (36.7%) or chronic end-stage liver failure (63.3%). Graft rejection occurred in 19 patients (63.3%); 6 pts required retransplantation for chronic rejection. Recurrence of initial disease was observed in 6 patients (20.0%), all of them with type 1 AIH, after a median time of 42 months, requiring retransplantation in 2 cases. Overall patient survival rates were 96.4%, 84.6%, 74.8%, 68.0%, 68.0%, 68.0% and 68.0% at 1, 5, 10, 15, 20, 25 and 30 years, respectively. Age at LT<1year (p<0.0001), LT for fulminant failure (p=0.023) and LT for type 2 AIH (p=0.049) were significant predictive factors of death.
Conclusion:
Long-term outcome after LT for pediatric autoimmune liver disease is impaired in patients with AIH because of consistent complications such as rejection and disease recurrence.
