[Autoimmune hepatitis: pediatric aspects]. / Epatite autoimmune. Aspetti pediatrici.

ABSTRACT

Autoimmune hepatitis (AIH), a liver inflammatory condition, is characterized by hypergammaglobulinemia, hypertransaminasemia, presence of autoantibody and periportal hepatitis revealed by histology. Usually it comes with acute onset and with bad prognosis. We describe recent updated literature for clinical manifestation and pathogenesis, principally referring to adult disease. We described the data obtained from a multicenter investigation (10 Italian center) included in the Gastroenterology and Hepatology Italian Society. The investigation collected 42 AIH cases in 18 years, this confirms that the disease is rare for pediatric age. There is a prevalence of females on males but not as important as in adults (F/M3/1). The average age at diagnosis was 7 + 4 years old. The diagnosis was always made based on observed symptoms excluding 2 cases with silent hypertransaminasemia. The most frequently observed autoantibody was SMA. In two cases, diagnosis was done without finding commonly investigated autoantibody. Prognosis is severe, in one case the hepatic transplantation was successful.