Based on serum autoantibody (Ab) profiles, autoimmune hepatitis (AIH) is classified as type 1 or type 2 (AIH-1/2). AIH can manifest in children as acute or chronic liver failure, or as cirrhotic AIH with or without overlap sclerosing cholangitis (SC). The purpose of this study was to examine demographics, presentation, and results in children across groups. A retrospective electronic file analysis was done at Texas Children’s Hospital on children with AIH who met conventional diagnostic criteria and had histologic confirmation, with de novo AIH following liver transplant (LT) excluded. Patients were identified and classified as AIH-1, AIH-2, ALF, CAIH, AIH-SC, or LT. There were 72 children with AIH-1, 19 with AIH-2, 13 with ALF, 25 with CAIH, and 14 with AIH-SC among the 91 children with AIH. AIH-1/2 was dominated by women and Hispanics. AIH-2 had a lower mean age in years than AIH-1. After one year of IS and the most current follow-up, both AIH-1/2 had low rates of remission. Twenty-two patients were treated with LT: 16 with AIH-1, 6 with AIH-2, 9 with ALF, and 13 with CAIH. One-year patient and graft survival rates were both 100%.

The epidemiology and clinical presentation of AIH-1 and -2 differed slightly. AIH-1 was linked to greater problems following LT. More information is needed to distinguish the two as distinct disease entities.