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Liver Transplantation for Autoimmune Hepatitis

  • Steven ZacksEmail author
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Abstract

Autoimmune Hepatitis (AIH) is a chronic, progressive, inflammatory, liver disease that responds to immunosuppressive therapy. Liver transplantation (LT) may be needed for AIH patients who present with acute liver failure without a prior diagnosis of AIH or in those who develop decompensated liver disease despite medical therapy. The proportion of all AIH that present as acute hepatitis that may progress to liver failure and need transplantation ranges from 20% to 75%. Approximately 2–3% of all pediatric and 4–6% of adult liver transplants in the US are for AIH. AIH can recur in the allograft with an incidence of recurrence between 8–12% at 1 year and 36–68% at 5 years. Recurrent AIH is characterized by non-organ specific autoantibodies, increased aminotransferases and immunoglobulin G (IgG), and typical histologic features of an interface hepatitis composed of peri-portal plasma cell infiltrates. De novo AIH is the development of features of classical AIH in the allograft of patients who have not been transplanted for AIH. Other names for this condition include ‘graft dysfunction mimicking AIH’ and ‘plasma cell hepatitis’. There is a need for uniform diagnostic criteria. Careful attention to excluding other causes of hepatitis is important in diagnosing de novo AIH. The cause of recurrent or de novo AIH is unknown. Several mechanisms have been implicated in this loss of self-tolerance including impaired thymic regulation, impaired activity of T regulatory cells, molecular mimicry, calcineurin inhibitors, glutathione-s transferase and genetic polymorphisms. While the phenotype of de novo AIH in pediatrics has been uniform, it has been more variable in adults, highlighting the need for uniform diagnostic criteria or scoring system post LT. Better understanding of the development of autoimmunity and its difference from classical rejection after LT will improve therapeutic strategies and outcomes. Experience in pediatric liver recipients suggests that immunosuppressive therapy used in AIH can successfully treat de novo AIH.

Keywords

Decompensated cirrhosis Fulminant hepatic failure Liver transplant Immunosuppressive therapy 

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Division of HepatologyCarolinas Medical CenterCharlotteUSA

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