Autoimmune liver disease.

PURPOSE OF REVIEW: This review aims to demonstrate how recent insights into disease behavior and mechanisms and the availability of new drugs can be assimilated into evolving concepts of diagnosis and treatment.

RECENT FINDINGS: Autoimmune hepatitis has a global distribution, and its clinical manifestations are similar in different regions. Concurrent immune diseases are common, and immunoglobulin A nephropathy may explain ascites in some patients. Subclinical celiac disease can cause cryptic liver dysfunction or be associated with autoimmune hepatitis. A fulminant presentation that is associated with de novo rather than exacerbated pre-existent disease is possible, and these patients may have centrilobular zone 3 necrosis. Bile duct injury as a background histologic finding should not change the diagnosis or therapy, and the clinical significance of autoantibodies can be determined only by examination of liver biopsy tissue. Molecular mimicry may be important in breaking self-tolerance, and a murine model based on DNA immunization with self-antigens supports this hypothesis. Corticosteroid therapy reduces or prevents hepatic fibrosis, and noninvasive techniques promise to facilitate the development of treatments that enhance this effect. Mycophenolate mofetil is a possible salvage therapy that requires clinical trial, and liver transplantation has a 5-year patient survival of 78% but is frequently followed by recurrent disease and acute rejection.

SUMMARY: Autoimmune hepatitis should be considered in all patients with acute or chronic liver disease. Therapy can reduce or prevent fibrosis. The new immunosuppressive drugs should undergo clinical trial before empiric use. Animal models promise to identify key pathogenic sites that can be targeted by therapies.