Hepatic sarcoidosis with portal hypertension. A report of seven cases with a review of the literature.
We have reviewed the clinical, histological and hemodynamic features of sarcoidosis complicated by portal hypertension in seven patients and in 40 previously reported cases. Young black patients of either sex and white females over 40 years were selectively affected. In 12 of these 47 patients, portal hypertension appeared to be a consequence of cirrhosis due to longstanding intrahepatic cholestasis; in white patients, this condition was clinically, histologically, and serologically indistinguishable from primary biliary cirrhosis. In most of the other patients, portal hypertension was the predominant and often the presenting symptom of hepatic sarcoidosis; in these patients portal hypertension was due to a presinusoidal block probably determined by portal granulomas, with or without superimposed sinusoidal block determined by fibrosis. Corticosteroids did not prevent the development of portal hypertension.