Outcomes in children who underwent transplantation for autoimmune hepatitis

Abstract

The outcomes of 113 children with autoimmune hepatitis (AIH), registered with Studies of Pediatric Liver Transplantation and who underwent transplantation between 1995 and 2006, were compared with those who underwent transplantation for other diagnoses (non-AIH). A total of 4.9% of liver transplants were for AIH; 81% of these patients had AIH type 1 and most underwent transplantation for complications of chronic disease (60%), the majority in females (72%). Transplantation for fulminant AIH was more common in males (52.5% versus 47.5% chronic; P ¼ 0.042). Patients with AIH differed from non-AIH patients by: age (13.0 6 0.4 versus 4.6 6 0.1 years; P < 0.0001), sex (64.6% female versus 52.9%; P ¼ 0.016), ethnicity (48.7% white versus 58.2%; P < 0.0001), initial immunosuppression (tacrolimus-based: 72.6% versus 62.6%; P ¼ 0.045; mycophenolate mofetil use: 31.0% versus 21.6%; P ¼ 0.02), and immunosuppression at 2 years after transplant (monotherapy: 51.9% versus 17.3%; P < 0.0001). Late (>3 months), but not steroid-resistant or chronic, rejection was more common in AIH (log-rank P ¼ 0.0015). The 5-year posttransplant survival for AIH was 86% (95% confidence interval: 73-93). Patient and graft survival, infectious and metabolic complications, and retransplantation rates did not differ between AIH and non-AIH groups. In conclusion, the higher risk for late acute rejection and greater degree of immunosuppression does not compromise outcomes of liver transplantation for AIH. Children who undergo transplantation for AIH in North America are typically female adolescents with complications of chronic AIH type 1 and include more children of African American or Latino American origin compared to the overall liver transplant population. These observations may inform detection, treatment, and surveillance strategies designed to reduce the progression of autoimmune hepatitis and subsequently, the need for transplantation.

Long-term medical management of the pediatric patient after liver transplantation: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation

“…In adults, the recurrence of AIH is more prevalent (approximately 22%‐41%) and more rapid (median interval ∼ 2 years) than the recurrence of PSC . The largest published series of children undergoing transplantation for AIH reported outcomes for 113 children enrolled in SPLIT . There were no differences between AIH children and non‐AIH children in patient or graft survival or in the incidence of first rejection up to 5 years after transplantation.…”

Liver Biopsy as a Useful Tool in the Management of Autoimmune Liver Diseases in Childhood

“…Liver transplantation is the treatment option of choice in end-stage AIH or in patients with acute severe/fulminant onset who do not respond to rescue immunosuppression. The 5-year posttransplant survival for these AIH-patients is 86% and patient and graft survival, infectious and metabolic complications, and retransplantation rates did not differ between AIH and non-AIH patients (Martin et al 2011). The higher risk for late acute rejection and the greater degree of immunosuppression needed does not compromise outcomes of liver transplantation for AIH.…”

Recurrence of autoimmune liver disease and inflammatory bowel disease after pediatric liver transplantation

“…(13) LT for Pediatric Autoimmune Liver Disease AIH accounts for 2%-5% of pediatric LTs performed in Europe and the United States. (15,16) Because AIH is a relatively rare condition, there are few studies reporting its outcome.…”