Characteristics and Outcome of three forms of Autoimmune Hepatitis in Iranian Children

Introduction To define the clinical features, biochemical and histological findings and outcome of three forms of autoimmune hepatitis.   Materials and Methods In a cross sectional study between November 2001 to January 2008 in Tehran and Mashhad university of medical sciences, 61 children who diagnosed as AIH (40 girls and 21 boys) have been analyzed for their clinical, serological, and histological data. Variables analyzed included age, sex, clinical presentation, hepatic function, immunoglobulins, autoimmunity markers, histology and clinical evolution. According to the auto-antibodies profile, AIH patients were classified as type I AIH (ANA or smooth-muscle antibodies or both positive), type II (anti-LKM-1 positive) and type III (antibody negative).   Results We analyzed data of 61 children with AIH. 51 patients (median age: 10 (0.7-14) years had anti-nuclear and/or smooth muscle antibody (ANA/ASMA or both), 5 (median age: 7 (2-8) years) had liver/kidney microsomal antibody (LKM-1). 5 patients had no detectable autoantibody. At presentation: 60% had jaundice or symptoms of acute hepatitis; LKM-1 positive were younger. Interface hepatitis was seen in 100% and fibrosis+/- cirrhosis were found in 70%. Frequency of AIH was 2:1 in girls. Type 1 was the most frequent diagnosis (83%) and was more frequent in older children. Relapse and treatment failure were common in type II.   Conclusion AIH may have different clinical presentation in children. Althout most of cases may have high inflammatory activity or cirrhosis, good response to treatment and remission may accur.