Primary Sclerosing Cholangitis

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Abstract
Primary sclerosing cholangitis was diagnosed in 6 patients (4 men and 2 women), with ages ranging from 33-71 yr. Four of them had ulcerative colitis, with an evolution from 8-21 yr, the other 2 had Crohn''s disease with 2 and 3 yr of evolution. Of the patients 66%, had pain in the right upper quadrant; 50% complained of itching, 33% presented with fever, jaundice, weight loss and hepatomegaly and 16% had splenomegaly. Of these patients 50%, were diagnosed as having Sjorgren''s syndrome. Alkaline phosphatase and .gamma.GT [.gamma.-glutamyl transpeptidase] were elevated in 100% of the cases; SGOT [serum glutamic oxaloacetic transaminase] and SGPT [serum glutamic pyruvic transaminase] 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. Two of them were diagnosed as having persistent chronic hepatitis and the remaining patient had hepatic fibrosis. ERCP [endoscopic retrograde cholangio-pancreatography] 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 and macrophages, and periductal fibrosis with obliteration of the canaliculae.