Primary sclerosing cholangitis: value of cholangiography in determining the prognosis.

We studied cholangiograms in 129 patients with primary sclerosing cholangitis (PSC) to determine if there was a correlation between any of the findings and the prognosis of the disease. The grade, length, and extent of strictures, the degree of bile duct dilatation, and the distribution of lesions were evaluated. Survival curves were generated to test the association of these radiologic signs with subsequent survival. High-grade intrahepatic duct strictures (greater than 75% narrowing) were associated with a 19% decrease in 3-year survival (p = .05) compared with lower-grade strictures. Diffuse intrahepatic strictures (involving greater than 25% of the ducts) were associated with a 16% decrease in 3-year survival (p = .012) compared with localized strictures. Statistically insignificant (p greater than .05) but measurable decreases in survival were observed with high-grade extrahepatic duct strictures, diffuse involvement of the extrahepatic ducts, long confluent strictures anywhere in the biliary tree, and marked dilatation of the intrahepatic ducts. In general, intrahepatic duct disease was found to have greater prognostic significance than extrahepatic duct disease. High-grade strictures and diffuse strictures of the intrahepatic ducts were found to be indicators of a poor prognosis in PSC and were more predictive of a poor prognosis than was extrahepatic duct disease.