title: Response to steroids in de novo autoimmune hepatitis after liver transplantation.
abstract: Graft dysfunction associated with autoimmune phenomena has been recently described in liver transplant recipients without previous autoimmune disease. However, the natural history, diagnostic criteria, and definitive therapeutic approach of de novo autoimmune hepatitis (de novo AIH) are poorly understood. We report 12 cases of de novo AIH 27.9 +/- 24.5 months after liver transplantation: the outcome of 7 patients treated with steroids is compared with a group of 5 nontreated patients. Nontreated patients lost the graft after 5.8 +/- 2.6 months from de novo AIH onset. All treated patients were alive after 48.4 +/- 14 (29-65) months from de novo AIH onset, and none of them lost the graft. However, 5 patients relapsed in relation to steroid tapering. All patients presented an atypical antiliver/kidney cytosolic autoantibody, associated to classical autoantibodies in 10 cases. Histological study showed several degrees of lobular necrosis and inflammatory infiltrate. HLA antigen frequencies and matching were compared with 2 control groups (16 orthotopic liver transplantation [LTX] patients without de novo AIH and 929 healthy blood donors); de novo AIH patients showed a higher prevalence of HLA-DR3 (54.5% vs. 25.9%, P =.04) than healthy controls, which was not observed in LTX patients without de novo AIH. In conclusion, this new disease should be included in the differential diagnosis of unexplained graft dysfunction. In addition, treatment with steroids results in a dramatically improved outcome. However, maintenance therapy is usually required.
authors.affiliation: Liver Transplantation Unit, Gastroenterology and Hepatology Department, Hospital General Universitario Gregorio Marañón, Universidad Complutense, Madrid, Spain. msalcedo@cvt.es
authors.forename: Magdalena
authors.initials: M
authors.lastname: Salcedo
authors.forename: Javier
authors.initials: J
authors.lastname: Vaquero
authors.forename: Rafael
authors.initials: R
authors.lastname: Bañares
authors.forename: Margarita
authors.initials: M
authors.lastname: Rodríguez-Mahou
authors.forename: Emilio
authors.initials: E
authors.lastname: Alvarez
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Vicario
authors.forename: Alicia
authors.initials: A
authors.lastname: Hernández-Albújar
authors.forename: José Luis R
authors.initials: JL
authors.lastname: Tíscar
authors.forename: Diego
authors.initials: D
authors.lastname: Rincón
authors.forename: Sonia
authors.initials: S
authors.lastname: Alonso
authors.forename: Alejandro
authors.initials: A
authors.lastname: De Diego
authors.forename: Gerardo
authors.initials: G
authors.lastname: Clemente
mesh_terms: D000328:Adult; D000914:Antibodies, Viral / Q000032:analysis; D005260:Female; D006680:HLA Antigens / Q000032:analysis / Q000145:classification; D019693:Hepatitis, Autoimmune / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008099:Liver / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D000914:Antibodies, Viral; D006680:HLA Antigens; D013256:Steroids