Autoimmune Liver Disease: Genes, Cells, and Therapy

Heiner Wedemeyer, MD and Michael P. Manns, MD


May 15, 2002

In This Article


Treatment options for autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are still limited and involve either nonspecific immunosuppression or ursodeoxycholic acid therapy. An understanding of the pathogenetic mechanisms of autoimmune liver disease is vital to the improvement of therapeutic options. A complex of events determines the development of immune-mediated liver disease. Indeed, in recent years it has become evident that the tight balance between autoaggression and tolerance can be affected by several internal and external factors. Activation of autoreactive T cells is believed to be the central event in autoimmunity and is thought to occur as a multistep process involving genetic susceptibility, environmental factors (ie, drugs, chemicals, and nutrients), presence of infection, and the local tissue microenvironment (Figure).

A number of interesting studies were presented during these meeting proceedings that investigated both basic and clinical aspects of autoimmune liver disease. This report discusses a select subset of these papers which we believe contribute significantly to our understanding, diagnosis, and therapy of AIH, PBC, and PSC.


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