Primary Sclerosing Cholangitis (PSC) and Inflammatory Bowel Disease (IBD)

1 Primary sclerosing cholangitis is a chronic cholestatic syndrome affecting both extrahepatic and intrahepatic bile ducts that is frequently progressive, leading to liver cirrhosis, portal hypertension, and eventually to end-stage liver disease. Primary sclerosing cholangitis is strongly associated with inflammatory bowel disease. The prevalence of inflammatory bowel disease (typically ulcerative colitis) among primary sclerosing cholangitis patients is approximately 70-80% while only 2-7.5% of patients with ulcerative colitis will develop primary sclerosing cholangitis. Primary sclerosing cholangitis is accompanied by an increased risk of liver failure, cholangiocarcinoma, and colorectal cancer. Cholangiography is considered the gold standard for the diagnosis of primary sclerosing cholangitis. It is noteworthy that medical, endoscopic, and surgical therapies do not convincingly alter disease progression in primary sclerosing cholangitis patients. Liver transplantation is currently the only available therapeutic modality for patients with end-stage primary sclerosing cholangitis. Abstract