Hepatic and extrahepatic manifestations in primary biliary cirrhosis

Abstract

Introduction: Primary Biliary Cirrhosis is a chronic progressive cholestatic disorder of unknown etiology, pathogenesis, characterized histologically by autoimmune inflammation of the biliary ducts interlobular septal and intrahepatic granulomatous destructive, associated with the presence of antimitochondrial antibody, cholestatic jaundice and pruritus. Primary biliary cirrhosis is ~ 0,6-2% of deaths from hepatic cirrhosis. Caution is required for diagnosis in women of average age with cutaneous pruritus, jaundice, steatorrhea and skin pigmentation. Purpose and objectives: The study of hepatic and extrahepatic manifestations in primary biliary cirrhosis. Materials and methods: Retrospective study of 40 patients hospitalized in Hepatology section of Republican Clinical Hospital was performed. 37 women (92.5%) and 3 men (7.5%) with a mean age of 47.7 years were included. Results: Typical hepatic manifestations of primary biliary cirrhosis were: hepatomegaly (34 patients; 85%), splenomegaly (21; 52.5%), skin jaundice (20; 50%), pruritus cutaneous (22; 55%) and asthenia (30; 75%). As extrahepatic manifestations were detected: osteoporosis (4 patients; 10%), arthralgia (7; 17.5%) and pneumosclerosis (7; 17.5%). Conditions associated with primary biliary cirrhosis were: thyroid diseases (6 patients; 15%), rheumatoid polyarthritis (4; 10%) and diabetes mellitus (5; 12.5 %). Biological markers associated with clinical features were: antimitochondrial antibodies (32 patients; 80%), antinuclear antibodies (8; 20%), rheumatoid factor (6; 15%) and cryoglobulins (3; 7.5%). Conclusion: In primary biliary cirrhosis classic symptoms (cutaneous pruritus, jaundice, hepatomegaly, splenomegaly and asthenia) may be associated with extrahepatic manifestations as: osteoporosis, arthralgia, pneumosclerosis, diabetes, rheumatoid arthritis and thyroid affectation.