Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome characterised by diffuse inflammation and fibrosis of both intra- and extra-hepatic bile ducts . Although PSC is most likely a multifactorial disease with genetic predisposition, its exact aetiology remains unknown. In 75% of cases, there is comorbidity with inflammatory bowel disease (IBD) such as ulcerative colitis (UC) and Crohn's disease. This underscores the role of immunological alterations in the pathophysiology of the disease. Mean age at diagnosis is 40 years, and men are affected about twice as often as women . PSC lacks a definitive medical therapy. Promising regimens are high doses of ursodeoxycholic acid alone or in combination with immunosuppression. Endoscopic treatment of biliary strictures is frequently needed in the late course of PSC disease. Repeated stenting may be an option in treating dominant strictures . Occasionally, surgical treatment of severe extrahepatic strictures is necessary. In general, however, most hepatobiliary surgeons are reluctant to perform such procedures, as they may complicate a future liver transplantation (Ltx) and reduce the chance of long-term survival following Ltx . PSC runs an unpredictable clinical course leading to liver cirrhosis and terminal liver disease in the majority of affected individuals.
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