Abatacept for Treatment of Recurrent or de Novo Autoimmune Hepatitis
Abatacept for Treatment of Recurrent or de Novo Autoimmune Hepatitis
The purpose of this study is to evaluate whether Orencia® (Abatacept) improves outcomes in
 liver transplant patients with recurrent or de novo AIH (autoimmune hepatitis) that has not
 responded to previous therapy. AIH that does not respond to steroids or conventional
 immunotherapy often affects young patients and leads to irreversible liver damage. There is
 currently no effective therapy for this condition.
Participants in this study will receive the study drug once a week for 6 months. If the study
 doctor feels the study drug has been beneficial, participants may receive additional study
 drug doses for up to an additional 6 months. Participants will continue on follow-up for 1
 year after stopping Abatacept.

 The risks related to participation in this study include potential side effects from the
 study drug include infusion-related reactions (reactions at the injection site), increase in
 respiratory adverse events, infections in patients with chronic obstructive pulmonary
 disease, risk of development of malignancies (cancer) or autoimmune disorders (which may make
 your current autoimmune disease worse or you can contract new autoimmune diseases). It is
 unknown if this is drug related. The study doctor will discuss any concerns about this with
 you prior to receiving treatment.

 There is the possibility that the participant may experience improvement in their medical
 condition from participation in this study. However, this benefit cannot be guaranteed. There
 may be no direct medical benefit to the participant due to their participation. The
 Investigator hopes that in the future the information learned from this study will benefit
 other people with this condition.
Inclusion Criteria for de novo AIH

 -  Age 18 or older

 -  Patients who have biopsy evidence of autoimmune liver disease following liver
 transplantation and a different pre-transplant etiology of liver disease will be
 eligible.

 -  Patients who have been treated with steroids or other immunosuppressive agents for at
 least a month and who have not responded either with respect to normalization of liver
 function tests or biopsy evidence of hepatitis will be eligible.

 -  Ability to provide signed and dated IRB approved written consent in accordance with
 regulatory and institutional guidelines prior to any protocol-related procedure.

 -  Women of child bearing potential agree to have pregnancy test at screening

 -  Males agree use of appropriate contraceptives during the active Orenia dosing period

 Inclusion Criteria for recurrent AIH

 -  Age 18 or older

 -  Patients who have biopsy evidence of autoimmune liver disease following liver
 transplantation and whose original etiology of liver disease was AIH will be eligible.

 -  Patients who have been treated with steroids or other immunosuppressive agents for at
 least a month and who have not responded either with respect to normalization of liver
 function tests or biopsy evidence of hepatitis will be eligible.

 -  Ability to provide signed and dated IRB approved written consent in accordance with
 regulatory and institutional guidelines prior to any protocol-related procedure.

 -  Women of child bearing potential agree to have pregnancy test at screening

 -  Males agree use of appropriate contraceptives during the active Orenia dosing period

 Exclusion Criteria:

 -  Active systemic infection

 -  Allergy to abatacept

 -  Known malignancy in the previous 2 years except for non-melanoma skin cancer

 -  Pregnancy or breast feeding

 -  Inability to commit to complete treatment protocol at Duke, as all procedures must be
 completed at Duke

 -  Prisoners or those who are compulsory detained

 -  Inability to read and understand English

 -  EBV seronegative (if not tested within 2 years prior to study enrollment, then testing
 will be done prior to study enrollment, results must be positive for study
 participation)