The syndrome of primary biliary cirrhosis.

Primary biliary cirrhosis, a chronic systemic disease, likely involves a disturbance in the immune process. The significant alterations in copper metabolism in this disorder are, we believe, related to progressive intrahepatic cholestasis and impairment in normal hepatobiliary copper clearance. A trial of D-penicillamine treatment suggests that this drug may benefit some patients. However, enthusiasm for the drug's use must be tempered by the fact that it may adversely affect 20% of patients; also, data regarding therapeutic effectiveness are not definitive. Therefore, we recommend using D-penicillamine for primary biliary cirrhosis only in control trials.