Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease in which inflammation and fibrosis produce multifocal strictures of the intrahepatic and/or extrahepatic bile ducts. Narrowed ducts impair bile flow, raise biliary pressure and promote further biliary injury. The course is slow but unpredictable: a person may feel well while strictures and fibrosis progress, or may present with pruritus, jaundice, abdominal pain, fever or complications of advanced liver disease. PSC is frequently associated with inflammatory bowel disease (IBD), which may be clinically silent. It also increases the risk of cholangiocarcinoma, gallbladder malignancy and, when IBD coexists, colorectal cancer. A useful clinical model is therefore broader than one abnormal liver test: recognize a cholestatic pattern, define the biliary anatomy, exclude secondary causes, look for associated IBD, and establish longitudinal surveillance. No medical treatment has been proven to stop or slow PSC progression. Care instead combines monitoring, treatment of symptoms and complications, endoscopic therapy for clinically relevant obstruction, and transplant referral for advanced disease. In Canada, an NP orders tests, prescribes, refers and performs procedures only within provincial or territorial legislation, individual competence,...