Clinicopathologic features of the syndrome of primary sclerosing cholangitis

摘要: Abstract We reviewed the histories of 50 patients diagnosed surgically and/or radiographically as having primary sclerosing cholangitis seen from 1970 through 1977. Patients were generally young males who presented with jaundice, pruritus, and hepatomegaly. Inflammatory bowel disease, usually chronic ulcerative colitis, was common (54%). A cholestatic biochemical profile ordinarily present, serologic markers conspicuously absent. Initiation endoscopic retrograde cholangiography in 1974 associated a doubling annual frequency diagnosis. Radiologic evidence both extra- intrahepatic duct involvement found (81%). Abnormal liver biopsies 34/36 showed cholestasis, portal or periportal hepatitis, cirrhosis. Hepatic copper levels elevated 12/12, mean level (244 ± 71 μg/g dry weight liver, SEM) comparable to that biliary Adequate follow-up data 39 13 died 5 108 mo after diagnosis, failure. Many (42%) received steroid therapy, without apparent benefit. In summary, diagnosis at our institution, particularly since advent cholangiography, is not rare. The disease characterized by male predominance, progressive cirrhosis, hepatic overload, failure, premature death