Pediatric Autoimmune Liver Disease

Abstract

Content available: Audio Recording Pediatric autoimmune liver diseases encompass a range of chronic immune-mediated conditions that include autoimmune hepatitis, sclerosing cholangitis, and posttransplant de novo autoimmune hepatitis. Autoimmune liver disease can present as an acute hepatitis or oftentimes more insidiously and is typically characterized by elevations of total immunoglobulin G and the presence of autoantibodies in the absence of metabolic or infectious etiologies. Improved awareness and understanding about the pathophysiology of these progressive conditions has led to an increasing recognition, although it remains unclear whether there has been a true increase in the prevalence of autoimmune liver disease. Early diagnosis with initiation of treatment can help prevent progression of hepatic parenchymal inflammation, but the bile duct disease in sclerosing cholangitis remains progressive. The goals of treatment include remission of inflammation followed by maintenance. In addition, given the chronic nature of these conditions, attention to the psychosocial impacts on children should be considered. Liver transplant remains a viable therapeutic option for end-stage autoimmune liver disease. The goal of this series of articles is to provide an update on our understanding of these conditions, along with current management strategies and considerations.