Immunosuppressive therapy allows recovery from liver failure in children with autoimmune hepatitis

Abstract

Background & aims: Patients with autoimmune hepatitis (AIH) frequently have liver failure (LF) at the time of diagnosis; their response to immunosuppressive therapy has not been thoroughly analyzed. We evaluated the outcomes of children with AIH and LF who received immunosuppressive therapy and analyzed predictors of liver function recovery.

Methods: We collected data from 237 children that had AIH between September 1996 and December 2008; 50 had LF (defined as prothrombin time <50%) and had not received prior treatment. Patients were treated with either 2 mg/kg/day prednisone at doses up to 60 mg/day (n = 13) or 1 mg/kg/day prednisone at doses up to 40 mg/day plus cyclosporine at blood levels of 200 ± 50 ng/mL (n = 37).

Results: Of the 50 patients studied, 45 (90%) achieved prothrombin time >50% in a median time of 24 days (range of 4-257 days); 93% of these patients achieved this within the first 90 days of treatment. Two of the 45 patients who responded to immunosuppression required liver transplantation because of complications related to portal hypertension, and 3 died because of infection. Three of the 5 nonresponders received liver transplants - 1 remained on the waiting list, and the other died because of central nervous system bleeding. Infection was the only independently associated significant factor that delayed recovery from LF (odds ratio = 7.7, 95% confidence interval, 1.5-40). Each therapeutic approach had similar efficacy.

Conclusions: Most pediatric patients with AIH recover after LF with immunosuppressive therapy; liver transplantation could be avoided or delayed. Infection was the most frequent cause of morbidity and mortality in these patients.