Autoimmune Liver Disease in Children - Abstract
Autoimmune liver disease in children encompasses autoimmune hepatitis (AIH), AIH overlapping with sclerosing cholangitis, drug induced AIH, AIH in syndromes, systemic disease and AIH after liver transplantation (LT). AIH is an uncommon progressive disease of the liver of unknown etiology affecting all ethnic groups and with female predilection. Characteristic clinical findings include elevated transaminases, elevated total Immunoglobulin G, positive autoantibodies and interface hepatitis on liver
histology, in the absence of other known causes of liver disease. A scoring system is available to enable diagnosis given there is no single pathognomonic feature for AIH. AIH is designated as type 1 when there is positivity for antinuclear antibody +/- smooth muscle antibody and type 2 when there is positivity for liver kidney microsomal antibody demonstrated. Clinical presentation can range from asymptomatic to acute
hepatitis like picture, jaundice, acute liver failure, and end-stage liver disease. AIH is characteristically treated with immunosuppression. Patients who have overlap with sclerosing cholangitis respond biochemically to ursodeoxycholic acid (UDCA), but effect on long-term outcome in children is unclear. LT is indicated in acute liver failure with encephalopathy or end-stage liver disease. AIH may recur after LT and also may occur denovo in a proportion of children transplanted for conditions other than AIH.