P0302 INTRAVENOUS METHYLPREDNISOLONE PULSE THERAPY FOR CHILDREN WITH AUTOIMMUNE HEPATITIS

Abstract

Introduction: Corticosteroids have been used as the first choice for autoimmune hepatitis(AIH), and have provided a high remission rate. However, high-dose or medium-dose corticosteroids frequently bring many side effects during long-term treatment, especially central obesity, and growth retardation in children. The major purpose of this study was to evaluate an efficacy of intravenous methylprednisolone pulse therapy (m-PSL) and the frequency of corticosteroid-related side effects in children with AIH, compared with oral corticosteroid therapy. Methods: During 2002 since 1991, 11 Japanese children (4 boys and 7 girls) with AIH were treated. The diagnosis of AIH was based on the diagnostic criteria and scoring system for AIH published in 1999. The initial symptoms developed at a mean age of 7.6 years (range, 2.0 to 14.8 years). In order to analyze the efficacy of therapy and corticosteroid-related side effects, patients were divided to 2 groups. Group 1 was consisted of 4 patients who were treated with conventional oral prednisone therapy without m-PSL. Group 2 consisted of 5 children who were treated with m-PSL as an induction therapy and maintained on oral prednisone. Two patients were excluded from this analysis because one patient was also complicated with autoimmune thrombocytopenia with administration of cyclophosphamide, and another patient already had liver cirrhosis at the time of diagnosis of AIHand could not achieve normal transaminase level. Results: Normalization of serum transaminase was noted for all patients within 15 to 617 days. The mean duration needed for normalization of transaminase was 301.3 days (range 18 to 617 days) in group 1, and 61.8 days (range 15 to 116 days) in group 2. The levels of transaminase in group 2 become normal earlier than in group 1. The hospitalization of group 2 (64.4 days) was clearly shorter than in group 1 (121.3 days). The most frequent side effects were obesity and growth retardation. The Z-score in group 1 tended to be lower than in group 2, and Kaup index in group 1 tended to be higher than in group 2. In all patients from group 1, Z-score decreased to less than zero. On the other hand, in 1 of 4 patients from group 2, the Z-score was lower than the mean level. Conclusion: The m-PSL can reduce negative effect of corticosteroids, especially growth retardation and obesity. In conclusion, m-PSL is a safe and effective therapy for children with AIH.