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.
