Primary sclerosing cholangitis

This code signifies a chronic, progressive cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, both intrahepatic and extrahepatic. It leads to strictures and dilation of the biliary tree, eventually resulting in cirrhosis, liver failure, and an increased risk of cholangiocarcinoma. The primary nature indicates that the cause is unknown and not secondary to another identifiable condition.

Use this code when documentation confirms a diagnosis of primary sclerosing cholangitis, typically established through imaging (e.g., MRCP, ERCP) showing characteristic multifocal stricturing and dilation of bile ducts, often accompanied by liver biopsy findings. This code is appropriate for initial diagnosis, ongoing management, and complications directly related to the underlying PSC.

• Ensure documentation clearly specifies "primary" sclerosing cholangitis to differentiate from secondary causes of sclerosing cholangitis.
• Code any associated complications, such as cholangiocarcinoma, cirrhosis, or portal hypertension, in addition to this primary diagnosis.
• Do not confuse with other types of cholangitis or cholecystitis; PSC has distinct diagnostic criteria.