Aim of the study:

This study was performed to investigate the hepatic expression of glucocorticoid receptors (GR) and 11b-hydroxysteroid dehydrogenase type 1 (11b-HSD1) in pediatric autoimmune hepatitis (AIH) patients and its relation to the steroid response.

Material and methods:

This study included 100 patients diagnosed with AIH on immunosuppressive therapy with different responses to treatment. The patients were subjected to full history taking and thorough clinical examination, laboratory investigations, abdominal ultrasound and liver biopsy for histopathological evaluation and assessment of the hepatic expression of GR and 11b-HSD1.

Results:

Out of the 100 cases, 82 cases showed a complete response, 11 a partial response and 7 cases were non-responders. The sex, age distribution and clinical presentation of the disease were comparable among the different response groups. Glucocorticoid receptors reactivity was significantly more intense in patients with a complete response than both patients with a partial response and non-responders. 11b-HSD intensity was higher in complete and partial responders in comparison with non-responders but without significance. The percentage of patients with a GR intensity score ≥ 200 was significantly higher in patients with a complete response than patients with a partial response and non-responders (p < 0.05). The GR intensity score had a significant positive correlation with intensity of 11b-HSD (r = 0.369, p < 0.0001).

Conclusions:

Glucocorticoid receptors expression was significantly variable in children with AIH and closely related to the response to therapy. However, the 11b-HSD expression was comparable between different response groups.