Title: Autoimmune Hepatitis: A Childhood Disease
Volume: 1
Issue: 1
Author(s): Giuseppe Maggiore and Marco Sciveres
Affiliation:
Keywords:
chronic liver disease, autoimmune hepatitis, interface hepatitis, immunosuppressive therapy, cyclosporine, liver transplantation
Abstract: Autoimmune hepatitis is a severe and unresolving inflammatory disease of the liver of unknown etiology carrying high morbidity and mortality. All ages and genders are concerned with a peak of incidence in girls in prepubertal age, even if the disease has been diagnosed as early as 6 months. Autoimmune hepatitis may be classified in two major subgroups on a presence of a specific set of autoantibodies: anti-smooth muscle mostly with anti-actin specificity and / or by antinuclear antibody in type 1 and anti-liver-kidney microsome and / or the anti-liver cytosol in type 2. The histological hallmark is “interface hepatitis”, with a mononuclear cell infiltrate in the portal tracts, variable degrees of necrosis, and progressive fibrosis. The disease follows a chronic but fluctuating course usually progressing to cirrhosis and liver failure. The most frequent type onset is similar to that of an acute viral hepatitis with acute liver failure in some patients; about a third of patients have an insidious onset with progressive fatigue and jaundice while 10-15% are asymptomatic and are accidentally discovered by the finding of hepatomegaly and / or an increase of serum aminotransferase activity. Corticosteroids alone or in conjunction with azathioprine are the treatment of choice inducing remission in over 90% of patients. An alternative therapeutic strategy is cyclosporine. Withdrawal of immunosuppression is associated with high risk of relapse. Liver transplantation manages patients with decompensated liver disease unresponsive to “rescue” medical treatment.
