Primaire scleroserende cholangitis

- Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown cause, with genetic predisposition in combination with environmental factors possibly playing a role.- The diagnosis is made on the basis of a liver enzyme profile indicating cholestasis and characteristic bile duct abnormalities in cholangiography or the liver biopsy after excluding other causes.- Approximately 80% of patients have concurrent inflammatory bowel disease (IBD), specifically ulcerative colitis in most patients.- PSC predisposes to hepatobiliary malignancies such as cholangiocarcinoma, gallbladder carcinoma and hepatocellular carcinoma, as well as to colorectal carcinoma in patients with concurrent IBD.- UDCA and endoscopic bile duct dilatation relieve symptoms and improve the liver enzyme profile.- Orthotopic liver transplantation is the only potentially curative therapy available