Long-term outcomes of de novo autoimmune hepatitis in pediatric liver transplant recipients

Udeme D. Ekong*, Patrick McKiernan, Mercedes Martinez, Steven Lobritto, Deirdre Kelly, Vicky L. Ng, Estella M. Alonso, Yaron Avitzur

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

The long-term course and outcome of DAIH is unknown. A retrospective multicenter study assessing associations and long-term consequences of DAIH developing in a transplanted allograft is presented. Children with DAIH were followed from diagnosis until death, re-LT, or transfer of care and for a minimum of 1 year. A total of 31 patients of 1833 (1.7%) LT were identified; 29 followed for a median of 7.1 years (range, 1.6-15); 52% had no rejection preceding diagnosis of DAIH. Transaminases fell following treatment with steroids and antimetabolites (ALT 108 vs 39 U/L (P=.002); AST 112 vs 52 U/L (P=.003); GGT 72 vs 36 U/L (P=.03), but this was not universally sustained. Transaminases >2X ULN observed in 38% of patients at last follow-up; commonly GGT, attributed to bile duct injury and ductopenia. Portal hypertension (PHT) was seen in four patients and associated with severe fibrosis and cirrhosis. Re-LT occurred in two patients for chronic rejection (CR) and uncontrolled PHT with gastrointestinal bleeding, respectively. No deaths from DAIH were reported. DAIH is an uncommon complication following pediatric LT requiring prolonged and augmented immunosuppression. It is associated with continued allograft dysfunction and may lead to bile duct injury, CR, and PHT necessitating re-LT.

Original languageEnglish (US)
Article numbere12945
JournalPediatric transplantation
Volume21
Issue number6
DOIs
StatePublished - Sep 2017

Keywords

  • allograft dysfunction
  • bile duct injury
  • chronic rejection
  • liver retransplantation
  • portal hypertension

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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