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Previous Volume 332:924-933 April 6, 1995 Number 14 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Primary sclerosing cholangitis, a chronic cholestatic liver disease of unknown cause, is characterized by ongoing inflammation, destruction, and fibrosis of intrahepatic and extrahepatic bile ducts.^1,2,3,4 Over time, bile ducts become irregularly narrowed and obliterated, and small intrahepatic ducts disappear. Focal bile-duct dilatation proximal to areas of stricture produces a characteristic beaded appearance on cholangiography.^5,6 Primary sclerosing cholangitis, although only about 1 percent as common as alcoholic liver disease,^7,8 is the fourth leading indication for liver transplantation in adults in the United States.⁹ The disease progresses silently, but relentlessly, in most patients and leads to cirrhosis, portal hypertension, and liver failure.^10,11

. . . [Full Text of this Article]

Pathological Features

Pathogenesis

Diagnosis

Clinical Features

Natural History

Treatment

Management of Chronic Cholestasis and Its Complications

Medical Therapy for Primary Sclerosing Cholangitis

            Corticosteroids

            Penicillamine

            Ursodiol

            Methotrexate

            Other Medical Agents

            Proctocolectomy

            Liver Transplantation

Conclusions

Source Information

From the Division of Gastroenterology, New England Medical Center, 750 Washington St., Boston, MA 02111, where reprint requests should be addressed to Dr. Kaplan.

References

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