Table 2.

Established Subtypes of Primary Sclerosing Cholangitis.^*

Subtype	Diagnostic Approach and Criteria	Cholangiographie Features	Histopathological Features	Management	Other Features
Classic	M RCP or ERCP with typical cholangio-graphic features; elevation of alkaline phosphatase level (more than doubled) for >6 mo; exclusion of causes of secondary sclerosing cholangitis	Affects small and large bile ducts	Mixed inflammatory-cell infiltrate, usually more intense around bile ducts; often nonspecific and nondiagnostic	Evaluate and treat coexisting conditions; endoscopic management of dominant stricture; liver transplantation for advanced disease	70–80% of patients have inflammatory bowel disease; increased risk of colon and gallbladder cancer, cholangiocarcinoma, and hepatocellular carcinoma
Small-duct	Liver biopsy; elevation of alkaline phosphatase level (more than doubled) for >6 mo; exclusion of causes of secondary sclerosing cholangitis	Affects only small bile ducts	Mixed inflammatory-cell infiltrate, usually more intense around bile ducts; often nonspecific and nondiagnostic	Evaluate and treat coexisting conditions; liver transplantation for advanced disease	May progress to classic subtype; associated with longer survival and less risk of cholangiocarcinoma than classic subtype
Associated with autoimmune hepatitis	Laboratory evidence of autoimmune hepatitis plus MRCP or ERCP findings of primary sclerosing cholangitis; exclusion of causes of secondary sclerosing cholangitis	Affects small and large bile ducts	Lymphoplasmacytic infiltrate, interface hepatitis	Same as for classic sub-type (see above); treatment for autoimmune hepatitis	Better prognosis than with classic subtype but worse prognosis than with autoimmune hepatitis alone

^*MRCP denotes magnetic resonance cholangiopancreatography.