Primary biliary cirrhosis. The experience in 33 consecutive cases Cirrosis biliar primaria. Experiencia en 33 casos consecutivos.

Abstract

Primary biliary cirrhosis is a chronic, progressive and often fatal cholestatic liver disease. We report clinical characteristics and follow up in 33 consecutive patients studied at a single university hospital during the last 10 years. 31 were female (94%) and the mean age was 51 +/- 2 years. At diagnosis, itching was present in 26 cases (78%). Association with autoimmune mediated diseases was frequent. Liver function tests showed marked cholestasis (alkaline phosphatase levels of 439 +/- 58 IU/l, range 90-1335). High antimitochondrial antibody titers and elevation of IgM levels were shown in all cases. According to liver biopsy findings, the diagnosis of primary biliary cirrhosis was an early one during the prospective phase of the study and was made in 8 +/- 1.4% of liver biopsies performed during this period. After a follow up of 27 +/- 5 months, 10 patients have died (30%). Our experience suggests that primary biliary cirrhosis is not an uncommon cause of chronic liver diseases in