Management of primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with fibrosis surrounding the intrahepatic and/or the extrahepatic bile ducts. PSC is characterized by progressive periductal obliterating fibrosis and bile duct strictures. In individual cases, PSC can have favorable prognosis but in most cases it is a progressive disorder which leads to liver related morbidity, mortality and the need for liver transplantation. In previous early cohorts median survival free of transplantation was 12 years whereas more recent studies indicated a median transplantation free survival of 18 years. More recently, patients with small-duct PSC, who have biochemical and histological features similar to other PSC patients but with a normal cholangiography have been shown to have a better prognosis than classic large-duct PSC. PSC is complicated by cholangiocarcinoma (CCA) which develops in 10-30% of PSC patients depending on the length of follow-up. The diagnosis of an early CCA in the setting of PSC is a major challenge and no consensus on screening strategies exists. No curative therapy for PSC is available at the current time except liver transplantation. The etiopathogenesis of PSC is unknown but the underlying pathophysiology of PSC is beyond the scope of this paper.