Long-term outcome of autoimmune hepatitis in children

Background and Aim: Autoimmune hepatitis (AIH) is a chronic disease of unknown etiology, which usually progresses to cirrhosis if not diagnosed and treated promptly. Data on long‐term follow up in children with AIH are scant. The aim of this study is to assess the long‐term outcome of autoimmune hepatitis in children with respect to clinical and laboratory features at presentation. Methods: Data were extracted from the medical records of patients presenting over a 28‐year period (1972–2000) to the Royal Children’s Hospital, Melbourne, Australia. Additional information was obtained by interviewing patients, and their current physicians. Of the 30 patients (22 females, mean age 9 years) identified, 18 had type I, three had type II, four had autoimmune–polyendocrinopathy syndrome type 1, one had infantile giant‐cell hepatitis associated with Coomb’s‐positive hemolytic anemia, and four were seronegative (antinuclear antibody (ANA), smooth muscle antibody (SMA) and liver–kidney microsomal antibody (LKM)). Results: Clinical features at presentation included hepatomegaly (86%), jaundice (66%) and splenomegaly (50%). Initial investigations revealed a median serum bilirubin level of 55 μmol/L (range 6–425), median aspartate aminotransferase level of 678 IU (range 70–2548), and abnormal clotting in 33% of patients. Liver biopsies were performed on all patients at presentation and 11 showed cirrhosis (36%). The mean follow‐up period was 10.0 ± 7.8 years with 43% being followed for > 10 years. Only two patients died and one required transplantation. Fourteen (50%) patients continue to be on low dose prednisolone with azathioprine, two (7%) are on prednisolone alone, and six (21%) are on no therapy. When the cirrhotic and non‐cirrhotic patients were compared, the albumin level at presentation was significantly lower in the cirrhotic group (P = 0.01). Of the patients who were cirrhotic at presentation, six (54%) remain compensated with a mean follow‐up period of 8 years. All 24 patients currently under follow up are engaged in age‐appropriate activities including school, part‐ or full‐time work. Conclusion: Autoimmune hepatitis has a favorable long‐term outcome with a transplant‐free survival rate of 90% over a mean period of 10.0 ± 7.8 years (range: 0.5–23), and a normal or near‐normal lifestyle irrespective of presenting clinical, laboratory or histological features.