Session Time: 6:00pm-7:00pm
Presentation Time: 6:00pm-7:00pm
Location: Hall C & D
*Purpose: Autoimmune Hepatitis (AIH) can be defined as type 1 and type 2 based on autoantibodies. Liver transplant (LT) may be indicated for chronic liver disease (CLF) or acute liver failure (ALF). The study aim was to compare demographics, presentation, lab values peri-LT, and treatment between type 1, type 2, ALF, and CLF.
*Methods: Retrospective electronic chart review was conducted for all children with AIH at Texas Children’s Hospital who had received a LT further divided into ALF/CLF and type 1/type 2 with de novo AIH excluded.
*Results: 98 patients with AIH were identified, of which 22 (22%) underwent LT. Patients with type 1 AIH were predominantly female (75%), whereas type 2 AIH patients were all female (100%). Patients with type 1 AIH had higher IgG levels peri-LT, increased transaminases, and were associated with a higher percentage of rejection (68.8% in Type 1 vs 16.7% in Type 2, p-value = 0.0288). Patients with type 2 AIH had lower platelets and a higher INR at LT compared to type 1. ALF was associated with the highest percentage of autoimmune comorbidity (44.4%), and almost all (88.9%) had progressive liver fibrosis on explant. ALF had lower platelets and higher INR at LT compared to CLF. Liver microsome Ab (LKM) titers in type 2 AIH decreased post-LT but did remain positive. 1-year patient survival was 100% for all groups. All patients were treated with tacrolimus post-LT and in AIH 1 93.8% were on a second immunosuppressant (azathioprine, mycophenolate, or sirolimus) vs. 66.7% of AIH 2. Long term steroids were used in 83.3% of AIH 2 vs. 31.3% AIH 1. Data is presented in table 1; median values are reported.
Table I. Patient Demographics, Lab Values, and Presentation.
|Type 1||Type 2||ALF||CLF|
|Age of Diagnosis||12||11||12.5||12|
|Ethnicity % Caucasian, Hispanic, African American, Other||25%, 31.1%, 25%, 18.9%||50%, 50%, 0%, 0%||22.2%, 44.4%, 11.1%, 22.2%||30.8%, 38.5%, 23.1%, 7.7%|
|Pre-LT Peak IgG||2240||1540||2240||1800|
|Post-LT Peak IgG||1340||561||909||1589|
|Advanced Fibrosis at LT||100%||83.3%||88.9%||100%|
|PICU vs. Home Prior to LT %||62.5%, 25%||16.7%, 50%||33.3%, 55.6%||61.5%, 15.4%|
|Vascular Complications Post-LT||18.8%||33.3%||44.4%||7.7%|
*Conclusions: Children requiring LT for AIH differ in ethnicity, clinical, and laboratory presentation. Rejection rate was highest in AIH 1, potentially related to lower use of long term steroids. There appears to be a higher rate of vascular complications post LT in AIH 2, likely due to hypercoagulability, though survival was excellent.
To cite this abstract in AMA style:Roepe IG, Goss JA, Miloh T. Liver Transplantation in Children with Type 1 and Type 2 Autoimmune Hepatitis [abstract]. Am J Transplant. 2019; 19 (suppl 3). https://atcmeetingabstracts.com/abstract/liver-transplantation-in-children-with-type-1-and-type-2-autoimmune-hepatitis/. Accessed April 10, 2020.
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