Autoimmune hepatitis.

N Engl J Med 2006;354:54-66. Copyright © 2006 Massachusetts Medical Society. A utoimmune hepatitis is a generally progressive, chronic hepatitis of unknown cause that occurs in children and adults of all ages. Occasionally, it has a fluctuating course, with periods of increased or decreased activity. The diagnosis is based on histologic abnormalities, characteristic clinical and biochemical findings, and abnormal levels of serum globulins, including autoantibodies. Since the first descriptions of this disorder more than 50 years ago,1 many labels have been applied, but “autoimmune hepatitis” has been accepted as the most appropriate and least redundant term.2,3 Variant, overlapping, or mixed forms of autoimmune hepatitis that share features with other putative autoimmune liver diseases, primary biliary cirrhosis, and primary sclerosing cholangitis occur as well. The distinctions among these disorders at present are necessarily descriptive. It remains important to distinguish autoimmune hepatitis from other forms of chronic hepatitis, because a high percentage of cases respond to antiinflammatory or immunosuppressive therapy, or both. Although appropriate management can prolong survival, improve the quality of life, and avoid the need for liver transplantation, considerable therapeutic challenges remain in the treatment of this disorder.4