Sclerosing Cholangitis— a Manifestation of Systemic Disease

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Abstract
THE RARE disease called sclerosing cholangitis has been described in detail only 22 times in the past 40 years. Most publications are concerned with the clinical characteristics, pathology, and therapy of the inflammatory lesion which involves the extrahepatic bile ducts. The clinical picture is characterized by jaundice due to biliary tract obstruction caused by a diffuse, inflammatory thickening of the ductal walls. The ducts have been described as "cord-like" and on palpation at surgery are often said to feel like "thrombosed blood vessels." The diffuse sclerosis of the extrahepatic bile ducts, without evidence of neoplasm by histologic examination, is the major abnormality in this disease. The pathologic process may involve the entire ductal system or may be limited to the common or hepatic ducts. The mortality in most reported series is quite high, ranging from 20% to 50%, but long term follow-up studies are lacking.1,2 Differentiation from benign stricture,

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