Primary Sclerosing Cholangitis in Children: Unique Aspects and Approaches

This review provides a comprehensive overview of pediatric autoimmune cholangiopathies, with particular emphasis on primary sclerosing cholangitis (PSC). It summarizes the epidemiology, clinical presentation, diagnostic evaluation, and natural history of these chronic immune-mediated liver diseases in children. Key aspects include the frequent overlap between PSC and autoimmune hepatitis, the strong association with inflammatory bowel disease, and current approaches to laboratory, imaging, and histological assessment. The challenges of disease monitoring and prognostication in pediatric PSC are highlighted. In addition, this review discusses current management strategies, advances in understanding disease pathogenesis, and ongoing efforts to develop effective disease-modifying therapies for affected children.