Diagnosing Autoimmune Hepatitis in Children: Is the International Autoimmune Hepatitis Group Scoring System Useful?

Background & Aims: In 1999, the International Autoimmune Hepatitis Group (IAIHG) modified a scoring system to differentiate adult patients with definite or probable autoimmune hepatitis (AIH) from those with other forms of chronic liver disease. We assessed the use of the scoring system in children. Methods: Twenty-eight pediatric patients with AIH and/or sclerosing cholangitis were reviewed. Clinical, laboratory, and histologic data were collected to score patients both before and after standard treatment. Results: There were 8 boys and 20 girls. The median age at diagnosis was 11 years (range, 2-16 years). Twenty-one of 28 children were diagnosed with AIH, 4 as isolated primary sclerosing cholangitis (PSC), and 3 as overlap syndrome. At presentation, 18 of 21 (86%) with AIH scored as definite AIH and 3 of 21 (14%) scored as probable. No patient clinically diagnosed as AIH scored as other. Seven of 28 patients had proven PSC. All patients with isolated PSC scored as other. The 3 with overlap syndrome scored as definite AIH. When the gamma-glutamyltranspeptidase (GGT) ratio was substituted for the alkaline phosphatase (ALP) ratio, 5 patients were reclassified from definite to probable AIH. Four of these 5 had an incomplete response to therapy, and 2 of 4 have confirmed overlap syndrome. Conclusions: The IAIHG scoring system has a use in children. Patients who fall into the other category should have cholangiographic imaging. Using the GGT ratio instead of the ALP ratio in the IAIHG score may improve the specificity for children, identifying those likely to have biliary disease. When GGT is used, patients classified as needing probable pretreatment should be considered for biliary imaging.