title: The syndrome of primary biliary cirrhosis.
abstract: 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.
mesh_terms: D004311:Double-Blind Method; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use
chemical_list: D010396:Penicillamine