Doenças hepáticas autoimunes em crianças e adolescentes

Abstract

The autoimmune liver diseases are a group of immunomediated disordes that targets the hepatocytes and bile ducts. Those diseases includes the autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC) and the association between these two conditions called overlap syndrome or autoimmune sclerosing cholangitis. AIH is characterized by elevated transaminase levels, autoantibodies and immunoglobulin G. According to the antibody identified, two types of AIH can be recognized: type I and type II. Positivity to antinuclear antibody and smooth muscle antibody defines type I AIH, which can affect both adults and children, while antibodies to liver-kidney microsome define type II, most common in younger children. Both types are greatly responsive to immunosuppressive therapy with corticoid and azathioprine. PSC, although, is a chronic cholestatic liver disease, of unknown etiology, characterized by progressive inflammation, fibrosis and stricture of intra and extra hepatic bile ducts. The diagnosis established by cholangiography, which revels areas of focal dilatations and stricture. Until this moment, there are no effective treatments beside the liver transplantation. This disease is associated with many complications other than cirrhosis, such as cholangiocarcinoma and cholangitis, and is associated with a higher incidence of inflammatory bowel diseases. This article presents a brief review of those two diseases in children and adolescents, emphasizing the clinical suspicion and the initial approach of the patient.