ABSTRACT
We analyzed 31 patients with a diagnosis of primary biliary cirrhosis, 29 of them males, aged 23 to 72 years. Liver biopsy was diagnostic in all showing initial findings of the disease in 5. Echotomography and cholangiography demonstrated a patent biliary, tract. Antimithocondrial antibodies were present in 94% of patients. Alkaline phosphatase and biliary acid levels were useful for diagnosis. Pruritus was present with varying intensity in all patients, with premenstural exacerbations in 5 females who had cholestasis of pregnancy or hepatitis caused by progestin drugs before developing cirrhosis. Recurrent urinary tract infection was present in 8 patients, osteosporis in 24, Sjogren's syndrome in 24 and Crest syndrome in 4. Survival ranged from 1 to 12 years, death being caused by rupted esophageal varices in 12 patients and by liver failure in 7. Persistance of pruritus and altered liver function tests after cholestasis of pregnancy or hepatitis caused by progestins should lead to investigation of biliary cirrhosis