Animal models for autoimmune hepatitis.
The liver is the target of adverse immune reactions in three putative autoimmune diseases: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). These three diseases can be distinguished by clinical, histological, and immunological features. However, especially on the level of specific antibody formation an overlap can occur, which sometimes complicates diagnosis. In this review, we will concentrate on autoimmune hepatitis and the current state of animal models for this severe disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration, hypergammaglobulinemia, and autoantibodies. The hallmark of type 2 AIH is the generation of autoantibodies of the LKM-1 type. The major target of these antibodies is the cytochrome P450 isoform 2D6 (CYP2D6). In the past several attempts have been made to develop a reliable animal model that reflects the persistent hepatic destruction that occurs in human AIH. However, most models were only successful in causing a transient form of hepatic damage and often used rather complex ways of disease induction.
Christen
Urs
U
Johann Wolfgang Goethe University, Frankfurt am Main, Germany. christen@med.uni-frankfurt.de
Holdener
Martin
M
Hintermann
Edith
E
D000818:Animals; D004195:Disease Models, Animal; D019693:Hepatitis, Autoimmune / Q000209:etiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D051379:Mice; D018448:Models, Immunological