Primary Sclerosing Cholangitis Epidemiology, Diagnosis, and Presentation

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease characterized by idiopathic inflammation and fibrosis of intra- and extrahepatic bile ducts.1 Many hypotheses have been proposed regarding the etiology of PSC, the most widely recognized of which is that PSC is an immune-mediated disease triggered by an environmental factor leading to hepatocyte injury and inflammation in genetically predisposed individuals.2-7 PSC is associated with other immune-mediated diseases in the same patient or first-degree relatives.8-10 Roughly 80% of patients with PSC have concomitant inflammatory bowel disease (IBD).10,11 Among patients with PSC and IBD, 80% have ulcerative colitis (UC), while the remaining patients have unspecified IBD or Crohn disease (CD).(12) The natural history of PSC is variable, but in most patients, progressive inflammation, fibrosis, and stricturing of the bile ducts result in cirrhosis and complications of liver disease. The median survival from diagnosis to liver transplantation or death from liver disease has been estimated to be 18 years.13 Multiple subpopulations within

Automated summary

Primary sclerosing cholangitis (PSC) is an immune-mediated liver disease characterized by inflammation and fibrosis of bile ducts. It is commonly associated with inflammatory bowel disease, especially ulcerative colitis. The natural history of the disease varies among patients, but most will develop cirrhosis.