Response to steroids in de novo autoimmune hepatitis after liver transplantation.
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.
Salcedo
Magdalena
M
Liver Transplantation Unit, Gastroenterology and Hepatology Department, Hospital General Universitario Gregorio Marañón, Universidad Complutense, Madrid, Spain. msalcedo@cvt.es
Vaquero
Javier
J
Bañares
Rafael
R
Rodríguez-Mahou
Margarita
M
Alvarez
Emilio
E
Vicario
Jose Luis
JL
Hernández-Albújar
Alicia
A
Tíscar
José Luis R
JL
Rincón
Diego
D
Alonso
Sonia
S
De Diego
Alejandro
A
Clemente
Gerardo
G
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*
D000914:Antibodies, Viral; D006680:HLA Antigens; D013256:Steroids