Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic liver disease that leads to chronic cholestasis, biliary strictures and liver fibrosis. It is considered an immune-mediated liver disease, although the exact aetiopathogenesis is not clear and there has been no clinical benefit from immunosuppression. Symptoms in PSC can be variable and include pruritus, abdominal pain, jaundice and fatigue. Diagnosis of PSC requires the exclusion of secondary causes of cholangiopathy and can usually be made on non-invasive imaging without the need for liver biopsy, which is usually reserved for variants. There is a close association with inflammatory bowel disease, which affects approximately two-thirds of patients, and all patients should undergo a screening colonoscopy at diagnosis. No currently licensed medications have proven prognostic benefit; the mainstay of management is prompt intervention for complications, such as acute cholangitis and dominant biliary strictures, and surveillance for progressive fibrosis, portal hypertension and cancers. There is a variable prognosis in PSC, but without curative medical options the median time from diagnosis to liver transplantation or death is 10–22 years. There is a significantly increased risk of hepatobiliary and colorectal malignancy in PSC; however, there is a limited role for cancer surveillance. Liver transplantation is associated with excellent outcomes but there is a risk of recurrent PSC after transplantation.