Treatment and outcomes of autoimmune hepatitis in children

Abstract

Introduction. Autoimmune hepatitis (AIH) is one of the auto­im­mune liver disorders recognized in children, along with autoimmune sclerosing cholangitis (ASC) and de novo AIH after liver transplant. This study aimed to as­sess the treatment in children with AIH, and to identify cor­re­la­tions with disease outcomes. Materials and method. In this retrospective study, we included 30 hospitalized chil­­dren with AIH, followed-up in our clinic over six years. All patients met the International Autoimmune Hepatitis Group’s simplified diagnostic criteria (IAIHG criteria). Results. Pediatric patients with AIH were aged between 4 months old and 17 years and 8 months old, with a mean age of 6.5 years old. There were 19 girls and 11 boys. Most pa­tients had an acute onset of the disease. Three patients de­ve­loped acute liver failure. All patients had elevated tran­sa­mi­na­ses and 16 patients had cholestasis. Most pa­tients had increased IgG levels. There were 20 children with type I AIH with SMA and ANA, and 10 children with type II AIH with anti-LKM-1 and anti-LC-1. All patients re­ceived prednisone from the diagnosis. Fifteen patients re­quired the addition of azathioprine. Full clinical recovery with a normal level of transaminases was achieved in 18 pa­tients. Complete remission was achieved in 10 patients. Most children had a favorable outcome, with only five pa­tients presenting cirrhosis. Two children died due to com­pli­ca­tions of autoimmune cirrhosis. Conclusions. The out­come of chil­dren with AIH treated with immediate im­mu­no­sup­pres­sive therapy is favorable, with good long-term survival rates and with a positive effect on the quality of life.