engleski

Cyclosporine Induced Biochemical Remission in Childhood Autoimmune Hepatitis

The conventional treatment of autoimmune hepatitis (AIH) in children, which includes prednisone alone or in combination with azathioprine, induces remission in most cases but is often associated with poorly tolerated side effects. To avoid the adverse effects, Alvarez et al. introduced an alternative treatment regimen, using cyclosporine A (CyA) as primary immunosuppression. We carried out a retrospective study to evaluate the efficacy and tolerance of CyA treatment in children and adolescents with AIH treated in our center. During the 2000–2010 period, nine children (6 female) aged 5–17.5 years were diagnosed with AIH according to established international criteria. Following the suggested protocol, CyA was administered orally and when the transaminases tended to normalize, the dose was adjusted to lower serum levels. Conversion to a low dose of prednisone and azathioprine was started after 6 months, with gradual tapering and discontinuation of CyA. All nine patients had elevated transaminases and gammaglobulin levels, with proven histological changes typical for AIH in 8 patients that underwent liver biopsy (in one patient biopsy was contraindicated due to the prolonged prothrombin time). Serum ANA/SMA autoantibodies were positive in all but one patient, who had positive anti-LKM1. Complete or near-complete and persistent normalization of transaminase activity was observed in 8/9 patients within the first 6 to 12 months. In one patient with partial response, an overlap syndrome was established. After ursodeoxycholic acid was added complete remission was observed. All patients had an excellent clinical course and histological improvement. During the long-term follow-up (1.5–9 yrs ; median 4.5 yrs), biochemical relapse occurred in one patient after discontinuation of maintenance corticosteroid dose. Despite the registered improvement, none of the patients fulfilled the criteria for therapy discontinuation, so all of them are still receiving maintenance doses of prednisone or azathioprine. The applied protocol allowed for the control of the liver inflammatory disease in all of our patients and protected them from the side effects related to steroid treatment. Side effects of CyA were minimal and were well tolerated.

autoimmune hepatitis ; cyclosporine ; remission ; steroid side effects ; childhood ; prednisone ; azathioprine

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