Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as yellow discoloration of the skin and eyes, itching, and abdominal pain.
The bile duct scarring that occurs in PSC narrows the ducts of the biliary tree and impedes the flow of bile to the intestines. Eventually, it can lead to cirrhosis of the liver and liver failure. PSC increases the risk of various cancers, including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma.[1][2] The underlying cause of PSC is unknown. Genetic susceptibility, immune system dysfunction, and abnormal composition of the gut flora may play a role.[3][4] This is further suggested by the observation that around 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.[5]
No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a liver transplant,[1] but disease recurrence can occur in 25-30% of cases.[6]
PSC is a rare disease and most commonly affects people with IBD.[2] About 3.0 to 7.5% of people with ulcerative colitis have PSC, and 80% of people with PSC have some form of IBD.[3] Diagnosis usually occurs in people in their 30s or 40s.[3] Individuals of Northern European ancestry are affected more often than people of Southern European or Asian descent.[2] Men are affected more often than women.[7] The disease was initially described in the mid-1800s, but was not fully characterized until the 1970s with the advent of improved medical-imaging techniques such as endoscopic retrograde cholangiopancreatography.[7]
^ abLazaridis KN, LaRusso NF (September 2016). "Primary Sclerosing Cholangitis". The New England Journal of Medicine (Review). 375 (12): 1161–1170. doi:10.1056/NEJMra1506330. PMC 5553912. PMID 27653566.
^ abcFolseraas T, Boberg KM (February 2016). "Cancer Risk and Surveillance in Primary Sclerosing Cholangitis". Clinics in Liver Disease. 20 (1): 79–98. doi:10.1016/j.cld.2015.08.014. PMID 26593292.
^ abcKummen M, Schrumpf E, Boberg KM (August 2013). "Liver abnormalities in bowel diseases". Best Practice & Research. Clinical Gastroenterology. 27 (4): 531–542. doi:10.1016/j.bpg.2013.06.013. PMID 24090940.
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