Long Term Outcome of De-Novo Autoimmune Hepatitis in Pediatric Recipients of Liver Transplantation.
1Hospital for Sick Children, Toronto, Canada
2Birmingham Children's Hospital, Birmingham, United Kingdom
3Morgan Stanley Children's Hospital, New York
4Lurie Children's Hospital, Chicago
5Yale New Haven Children's Hospital, New Haven.
Meeting: 2016 American Transplant Congress
Abstract number: 357
Keywords: Hepatitis, Liver transplantation, Outcome, Pediatric
Session Information
Session Name: Concurrent Session: Pediatric Liver Transplant
Session Type: Concurrent Session
Date: Monday, June 13, 2016
Session Time: 4:30pm-6:00pm
Presentation Time: 5:06pm-5:18pm
Location: Room 206
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).
To cite this abstract in AMA style:
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. [abstract]. Am J Transplant. 2016; 16 (suppl 3). https://atcmeetingabstracts.com/abstract/long-term-outcome-of-de-novo-autoimmune-hepatitis-in-pediatric-recipients-of-liver-transplantation/. Accessed December 5, 2024.« Back to 2016 American Transplant Congress