Primary sclerosing cholangitis and inflammatory bowel disease in children: a modern view of the problem

Primary sclerosing cholangitis (PSC) is a progressive, chronic liver disease characterized by destruction of the intra- and extrahepatic bile ducts. Much remains unknown about primary sclerosing cholangitis, including its underlying cause. However, available data suggest a multifactorial etiology, where the interaction of a combination of genetic predisposition, environmental factors, gut microbiome imbalance, and immune dysregulation triggers activation of cholangiocytes and peribiliary myofibroblasts that function in concert to cause progressive fibroinflammatory destruction of the bile ducts. This article discusses the association of primary sclerosing cholangitis and inflammatory bowel disease and diagnostic methods for these conditions.