Primary sclerosing cholangitis (PSC)

Abstract

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease of unknown etiology. It is characterized by diffuse inflammation and fibrosis of bile ducts leading to strictures primarily in large- and medium-sized ducts of the biliary tree (fibro-obliterative cholangiopathy) [1, 2]. The cause is presumably immune-mediated and it is frequently associated with inflammatory bowel disease (IBD) with peculiar features (PSC-IBD). The progressive destruction of bile ducts may lead to liver cirrhosis requiring liver transplantation. In addition, episodes of recurrent cholangitis and hepatobiliary/colorectal malignancies are frequently complicating the clinical course of PSC [3, 4]. Although the characterization and understanding of the disease has improved substantially since its first descriptions in the mid-nineteenth century [5], effective medical therapy is still lacking and this disorder still represents a potentially severe disease with poor prognosis. So far no established pharmacological therapy improving survival of PSC exists, and ursodeoxycholic acid (UDCA) in combination with endoscopic interventions of dominant strictures is presently widely applied in clinical practice. Liver transplantation is the only established treatment of PSC in patients with end-stage liver disease, recurrent cholangitis, or high-grade cholangiocyte dysplasia.