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July 25, 1977

Chronic Intrahepatic Cholestasis With Granulomas and Biliary Cirrhosis: Enigmatic Disease and Therapeutic Dilemma

Author Affiliations

From the Section of Gastroenterology, New York Medical College-Westchester County Medical Center, New York.

JAMA. 1977;238(4):337-338. doi:10.1001/jama.1977.03280040057024

CHRONIC intrahepatic cholestasis, a syndrome characterized by jaundice, pruritus, and hepatomegaly, may occur as a result of a variety of causes. When associated with hepatic granulomas, many cases are due to adverse drug reactions, Hodgkin's disease, Crohn's disease, and postnecrotic cirrhosis, and these are relatively easily distinguished. Infections from viral agents, brucellosis and Q fever, and fungi may be excluded as causes by appropriate tests. Tuberculosis may be difficult to prove at times.

Primary biliary cirrhosis, which also is seen generally as chronic intrahepatic cholestasis, may be closely mimicked by the cholestasis that has been recently noted to rarely occur in patients with sarcoidosis.1,2 Advanced states of the two diseases may be indistinguishable, as exemplified by the patient described here.

Report of a Case  A 62-year-old woman was admitted to Westchester County Medical Center on April 23, 1976, for investigation of pruritus and elevated serum alkaline phosphatase values.The patient,