Autoimmune hepatitis (AIH) comprises a relatively diverse group of liver diseases associated with autoantibody formation (Figure 1) which are thought to occur as a result of an uncontrolled, self-directed inflammatory attack on hepatocytes or bile ducts. The immune injury commonly leads to rapidly progressive liver disease, progressive fibrosis, and ultimately cirrhosis. The cause of autoimmune liver disease is not known, but factors that influence the development of clinical disease include genetic predisposition, prior liver injury caused by viruses, environmental exposure to toxins, and, possibly, ongoing infectious factors, including viruses.

The incidence and prevalence of the forms of autoimmune liver diseases remain to be defined worldwide, although information is available for distinct ethnic populations.<sup>27 Berdal J.E., Ebbesen J., Rydning A. [Incidence and prevalence of autoimmune liver diseases] Tidsskr Nor Laegeforen 1998 ;  118 : 4517-4519

Cliquez ici pour aller à la section Références</sup> The point prevalence of autoimmune liver disease ranges from 13 to 20/100,000 population and the incidence from 1 to 2/100,000 in a Norwegian population.<sup>35 Boberg K.M., Aadland E., Jahnsen J. , et al. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population Scand J Gastroenterol 1998 ;  33 : 99-103

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Improving the understanding of autoimmune liver disease will allow physicians and other medical practitioners to make decisions about the proper type of therapy to prevent progressive liver disease and may ultimately eliminate death from liver failure and the need for liver transplantation. Other focuses of therapy include improving quality of life preventing primary septic complications caused by biliary strictures (in primary sclerosing cholangitis) and infectious complications of immune suppression.

Serologic assays, biochemical tests, histologic criteria, and imaging tests have provided the basis for the diagnosis of autoimmune liver disease. Recent advances in serologic testing, genetic studies, and immunohistochemistry may provide more rapid and accurate diagnosis of patients with autoimmune liver disease and may also guide the cause of immunosuppressive therapy.

Infectious causes of autoimmune liver disease have been proposed, with prior viral hepatitis A, B, or C, cytomegalovirus (CMV), paramyxovirus, and most recently retrovirus infections suggested as possibilities.<sup>108 Manns M.P., Obermayer-Straub P. Viral induction of autoimmunity: Mechanisms and examples in hepatology J Viral Hepat 1997 ;  4 (suppl 2) : 42-47  [cross-ref]

Cliquez ici pour aller à la section Références, 111 Mason A., Xu L., Guo L. , et al. Detection of retroviral antibodies in primary biliary cirrhosis and other idiopathic biliary disorders Lancet 1998 ;  351 : 1620-1624  [cross-ref]

Cliquez ici pour aller à la section Références</sup> The development of recurrent AIH, primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) in liver transplant recipients provides strong support for an infectious origin for of these disorders. If environmental agents are found to be the cause or a component of disease activity and progression, advances in antibiotic and antiviral therapies may also delay or prevent progressive liver disease.