[Autoimmune hepatitis in children]

Autoimmune hepatitis is a rare chronic disease of unknown aetiology with female predominance, circulating autoantibodies, hypergammaglobulinaemia, typical major human leukocyte antigen types (HLA A1/B8/DR3) and a good response to immunosuppressive therapy. It seems that the main features in autoimmune hepatitis are a primary or secondary defective suppressor-T-cell function. Recently, we are realizing the recommendations of the International Autoimmune Hepatitis Study Group, using a combination of clinical, biochemical and serological features.

Two hundred and twenty one patients with chronic liver disease were studied. In this study, 221 consecutive percutaneous liver biopsies were performed, with a Menghini needle, during a 5-year period. All children were under 18 years.

We found 17 patients with autoimmune hepatitis (7.7%) among children with chronic liver disease. In summary, final diagnosis was established in 16 patients (94%). Using minimum required (main) parameters final diagnosis was established in 10 patients (59%). We found no significant differences between histologic parameters and response to therapy. Our patients with autoimmune hepatitis were predominantly females (3.25:1) with antibodies to smooth muscle (59%). The main histologic findings were bridging necrosis, rosetting of the liver cells and plasma cell infiltration (83%). In two cases we found granulomas and in one case centrilobular necrosis.

Over a period of 5 years, the diagnosis of autoimmune hepatitis was established in 17 children. This is similar to the findings published in other paediatric studies [7, 8]. Patients were predominantly females as in the most recent studies [10, 11, 13-15]. Our results regarding the validation of the scoring system showed that we found no significant differences between histologic parameters and response to therapy [16-18]. Histologic findings were not pathognomonic but suggestive of autoimmune hepatitis diagnosis [1, 4, 8, 15-18]. The remission rate induced by the initial therapy was 88%, but in 53% of cases the disease was reactivated [3, 4, 11, 14, 15].