Primary sclerosing cholangitis.

Primary sclerosing cholangitis was diagnosed in 6 patients (4 men and 2 women), with ages ranging from 33 to 71 years. Four of them had ulcerative colitis, with an evolution from 8 to 21 years, the other two had Crohn's disease with 2 and 3 years of evolution. 66% of the patients had pain in the right upper quadrant; 50% complained of itching, 33% presented with fever, jaundice, weight loss and hepatomegaly and 16% had splenomegaly. 50% of these patients were diagnosed as having Sjörgren's syndrome. Alkaline phosphatase and gamma GT were elevated in 100% of the cases; SGOT and SGPT were slightly elevated in all cases; bilirubin was elevated only in 50% of the cases. Cholelithiasis was found in 2 patients with Crohn's disease and 1 patient with ulcerative colitis. All were subjected to laparoscopy. 2 of them were diagnosed as having persistent chronic hepatitis and the remaining patient had hepatic fibrosis. ERCP revealed alterations in the intrahepatic ducts and there was stenosis of the end of the common bile duct in one of them. The most frequent histological findings were periportal reactions with lymphocytes, plasma cells of macrophages, and periductal fibrosis with obliteration of the canaliculae.