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

Abstract

Though extensive research has been performed on primary biliary cirrhosis (PBC) in Caucasian patients, little is known about the disease in the Asian population. 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. 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 < or =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. 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.