Objective: To document the natural history of asymptomatic primary biliary cirrhosis and identify prognostic features that would predict the development of symptomatic disease.
Methods: A retrospective chart review of all patients with abnormal liver biochemical tests and antimitochondrial antibody-positive, liver biopsy-compatible primary biliary cirrhosis who were seen in a single tertiary care center between 1983 and 1994 was performed. Statistical analysis using Cox regression was employed to compare survival of the study population with an age- and gender-matched control population and to identify potential prognostic variables.
Results: Ninety-one patients were included. Median age at presentation was 53.2 yr. Ninety percent were female. Median follow up was 61.2 months (range 7-206 months). Thirty-six percent (33 patients) became symptomatic with 11% (10 patients) progressing to death or liver transplant. Median predicted length of survival from onset of disease for the entire cohort was 14 yr. Patient survival was less than that predicted for an age- and gender-matched control population (p < 0.05). Univariate and multivariate analysis on a broad spectrum of clinical, biochemical, and histological features at the time of initial presentation failed to reveal any prognostic variables that would distinguish those who would become symptomatic from those who would remain symptom-free. Specifically, three primary variables of interest (associated autoimmune disorders, hepatomegaly, and histological stage) were not found to predict prognosis.
Conclusion: Patients who present with asymptomatic primary biliary cirrhosis have a shorter life span than the general population. Presently, there are no prognostic features that identify the patients who will develop progressive disease from those who will remain symptom-free. Therefore, treatment should be offered to all patients.