Asymptomatic primary biliary cirrhosis. A progress report on long-term follow-up and natural history.
Thirty-six patients presenting with asymptomatic primary biliary cirrhosis have been followed for a median period of 11.4 yr, extending by 5 yr a previously reported median follow-up study of 6 yr. Life table survival analysis indicates that the overall survival of this subgroup of patients with primary biliary cirrhosis continues to remain similar to that of the general population (p = 0.91). Over this period, 15 patients developed symptoms and 8 patients died, 6 from liver disease; 21 patients remained in an asymptomatic state. Portal granulomas on initial liver biopsy were the only finding that correlated with a normal survival and a continued asymptomatic state (p = 0.03). In contrast, associated autoimmune disorders (thyroiditis, sicca syndrome, CRST syndrome, Raynaud's phenomenon) correlated with decreased survival (p = 0.01). No other clinical, laboratory, or histologic features correlated with survival or the development of symptoms. This extended follow-up study (median 11.4 yr) indicates that many patients with asymptomatic primary biliary cirrhosis have a benign outcome. Although 42% developed signs or symptoms of progressive disease at variable times up to 14 yr from presentation, the group survival remained similar to the general population.
D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000401:mortality; D001663:Bilirubin / Q000097:blood; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006099:Granuloma / Q000401:mortality; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged
D001663:Bilirubin