Primary sclerosing cholangitis (PSC) is a rare, idiopathic, heterogeneous, chronic, cholestatic liver disease characterised by inflammation and fibrosis of both the intrahepatic and extrahepatic bile ducts, leading to the formation of multifocal bile duct strictures.54-56
There is a close association with inflammatory bowel disease, which is present in the majority of patients.57
PSC eventually progresses to cirrhosis, portal hypertension and hepatic decompensation in the majority of patients.54,57
Patients can be asymptomatic at presentation or they may present with pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. PSC is associated with a high risk of cholangiocarcinoma in adults.57 In children, it is rarer, but a few cases have been reported. 61
PSC is relatively rare, affecting fewer than 200,000 individuals in the USA and less than 250,000 in the EU, or approximately 5 per 10,000 Europeans.57 Although PSC is increasingly diagnosed early, this has not led to improved outcomes.55
The causes and pathogenesis of PSC are unclear although it is thought that both genetic and environmental risk factors contribute its development and progression.56
Diagnosis
54. Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010;51:660-78.
55. Dyson JK, Beuers U, Jones DEJ, Lohse AW, Hudson M. Primary sclerosing cholangitis. Lancet 2018;391:2547-59.
56. Lazaridis KN, LaRusso NF. Primary Sclerosing Cholangitis. N Engl J Med 2016;375:1161-70.
57. Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis – a comprehensive review. J Hepatol 2017;67:1298-323.
Those requiring urgent treatment:
Biliary atresia
