Background: De-novo autoimmune hepatitis (DAIH) is a chronic immune mediated graft disorder seen following liver transplantation (LT). The long-term course and outcome of DAIH is largely unknown. A retrospective multi-center study assessing these aspects is presented.

Methods: Children with DAIH were followed from diagnosis until December 31, 2012, death, re-LT or transfer of care and for a minimum of 1 year. DAIH was diagnosed based on the Banff working group recommendations (Hepatology 2006). Long-term outcome data were collected in 5 LT centers.

Results: Thirty-one patients (58% females) with DAIH out of 1833 (1.7%) LT were identified. Follow up data was available for 29 patients. Seventeen (59%) patients underwent LT for biliary atresia, 5 for acute liver failure and 7 for other etiologies at a median age of 1.5 years (range:0.2-12.3). At least one episode of acute rejection was diagnosed in 14 (48%) patients before the diagnosis of DAIH. DAIH was diagnosed at a median of 5.3 years (range:1.2-14.9) post LT. Median ALT at diagnosis was 108 IU/L (IQR:40-216), GGT 72 IU/L (IQR:23-173), IgG 16.7 g/l (IQR:16.2-19.1) and total bilirubin 0.7 mg/dl (IQR:0.5-0.9). Prednisone at a mean dose of 0.84±0.66mg/kg was initiated in 21 patients and azathioprine in 14. Patients were followed for a median of 7.1 years (range:1.6-15). Immunosuppressive therapy at the last visit was calcineurin inhibitor based in 27 patients. Eighteen (62%) patients were maintained on prednisone at a median dose of 0.06 mg/kg and 21 (72%) on either azathioprine or mycophenolate mofetil. Exacerbation of DAIH was diagnosed in 17 patients during the follow up period. Re-LT due to portal/hepatic vein pathologies in the presence of DAIH was required in 2 patients. One patient died from a non-DAIH related pathology. Portal hypertension (PHT) secondary to DAIH was documented in an additional 2 patients. Abnormal liver enzymes (>2X ULN) were detected in 10 (38%) patients at their last follow up, most commonly GGT.

Conclusions: DAIH is an uncommon chronic complication following pediatric LT. It requires prolonged and augmented immunosuppressive therapy, is associated with continued mild graft dysfunction and very rarely it may lead to PHT but not to graft failure.

CITATION INFORMATION: Avitzur Y, McKiernan P, Martinez M, Lobritto S, Kelly D, Ng V, Alonso E, Ekong U. Long Term Outcome of De-Novo Autoimmune Hepatitis in Pediatric Recipients of Liver Transplantation. Am J Transplant. 2016;16 (suppl 3).

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