Primary sclerosing cholangitis -up to date-

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of unknown aetiology, which is characterized by the progressive stenosis and destruction of the bile ducts due to diffuse inflammation and fibrosis, and which eventually results in cirrhosis and liver failure. Inflammatory bowel diseases (IBD) are frequently noticed as comorbidities and inflammation at intestinal mucosa and abnormal bacterial flora are supposed to be involved with etiology of PSC. Onion skin lesion is a characteristic histological feature but rare to be seen. Therefore cholangiographic findings including beaded appearance and pruned tree appearance is diagnostic. Unfortunately, despite the severity of this disease, no standardized medical treatment is available currently, and although autoimmune reactions are believed to play a role in PSC pathogenesis, immunosuppressive therapy has not been effective. As a result, the long-term outcome for patients with PSC is unfavourable and is characterized by a high mortality rate, with liver transplantation (LT) being the only option for patients with advanced PSC.