Animal models of autoimmune liver diseases

Abstract

The term autoimmune liver diseases in its common use comprises three types of disorders, namely, autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), as well as their overlap syndromes. Previous reports (1,2) indicated an incidence (identified patients) of 1 to 2:10.000 for AIH, 1:10.000 for PBC, and 0.5:10.000 for PSC. However, there is a pronounced probability for significantly higher case numbers of unidentified autoimmune liver diseases owing to low severity of symptoms in the early stages, especially for PBC and PSC and also most of AIH cases. There is a predisposition of women to the development of AIH (3-4:1 female/ male patients) and PBC (9:1), whereas PSC predominantly affects men (1:2). © 2007 Humana Press Inc.