What Do I Need to Know About Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis

Abstract

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are rare progressive disorders that can ultimately lead to a host of complications, including liver cirrhosis. The diagnosis is unlikely to be made in the acute care setting, but should be considered in any patient with progressive or episodic cholestasis that cannot be attributed to another cause. Both conditions have unique features, including gender predominance, associated conditions, and long-term complications. Acute care providers should be aware of the treatment options for cholestatic pruritus, as well as the association of PSC with cholangiocarcinoma and colorectal cancer. Providers should evaluate the patient with PBC or PSC for signs and symptoms of cholangitis. They should also evaluate for complications or sequelae of cirrhosis (including hepatic encephalopathy, spontaneous bacterial peritonitis, and gastrointestinal bleeding) in patients with end-stage liver disease secondary to PSC or PBC.