Recent Insights into Pediatric Primary Sclerosing Cholangitis

Abstract

This article reviews recent literature on the pathogenesis, presentation, diagnosis, comorbidities, natural history, and management of pediatric primary sclerosing cholangitis (PSC). The authors shed light on the role of genetic and environmental factors in PSC, although recognize the limitations in the understanding of PSC pathogenesis. They reflect on presenting disease phenotypes, including the association with inflammatory bowel disease and frequent histologic presence of autoimmune hepatitis features. The current lack of effective medications is discussed, and disease complications and prognosis are described. Finally, the authors highlight available evidence while acknowledging the paucity of prospective pediatric data.

Keywords: Autoimmune sclerosing cholangitis; Immune-mediated liver disease; Pediatric liver disease; Pediatric sclerosing cholangitis; Primary sclerosing cholangitis.