[Autoimmune hepatitis in children].

UNLABELLED: 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.

METHODS

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.

RESULTS

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.

DISCUSSION AND

CONCLUSION

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].