A retrospective study on clinical features and prognostic factors of biopsy-proven primary biliary cirrhosis in Chinese patients

OBJECTIVES: Though extensive research has been performed on primary biliary cirrhosis (PBC) in Caucasian patients, little is known about the disease in the Asian population. PATIENTS AND METHODS: This was a retrospective study of Chinese patients with biopsy-proven PBC. Electronic records of results from all liver biopsies (n = 1,021) performed between January 1996 and April 2004, together with records of patients labeled as "biliary cirrhosis," were retrieved. Patients with biopsy-proven PBC were identified, and their medical notes were reviewed. The demographic, clinical, biochemical, and histological parameters of these patients were analyzed for mortality predictors. RESULTS: Thirty-nine patients with biopsy-proven PBC and a median follow-up of 44 (range: 5-114) months were identified. Twelve patients (30.8%) were asymptomatic at diagnosis. The patients were approximately equally divided into one-thirds at stages I, II, and III of the histological disease. Hepatic decompensation or hepatocellular carcinoma developed in 14 (35.9%) patients during the follow-up period. The overall 5-yr survival probability was 81.4%. Hypoalbuminemia was found to be the only independent predictor of mortality on multivariate analysis (hazard ratio = 0.50 per 1 g/L increase, 95% CI 0.30-0.84, p= 0.008). Using the median serum albumin level as the cutoff, the 5-yr survival probability was significantly higher for patients with serum albumin levels > 35 g/L than for those with serum albumin levels <= 35 g/L (100% vs 69%, p= 0.007). No significant difference was found when baseline serum albumin was compared with the Mayo Risk Score and the model for end-stage liver disease (MELD) score for prediction of patient survival (p= 0.68) and death (p= 0.12) at 5 yr. CONCLUSIONS: In this longitudinal cohort study of biopsy-proven PBC with up to 9 yr of follow-up, we found that Chinese patients with PBC had significant morbidity and mortality. Hypoalbuminemia at presentation was an independent and strong predictor of mortality.