Idiopathic sclerosing cholangitis. Pathophysiologic observations.

Selective hepatic arteriography and umbilical portography in a patient with idiopathic sclerosing cholangitis revealed uneven distribution of blood flow through the liver, the majority of the contrast media being demonstrated in the central portions of the liver. Mild portal hypertension was present. The stenotic biliary tree offered no unusual resistance to the passage of dye from the main biliary radicals into the duodenum. T tube drainage dramatically relieved pruritus, but clinical improvement with weight gain, disappearance of jaundice and reduction of portal pressure did not occur until after the administration of steroids. After one year of steroid therapy, liver biopsy showed a reduction in the periportal inflammatory reaction and lessening of the diffuse stenosis of the biliary tree. The relationship of these findings to current pathophysiologic hypotheses concerning sclerosing cholangitis is discussed.