OBJECTIVE: To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease. METHODS: General status, clinical manifestations and laboratory findings of 45 patients with PBC were reviewed. RESULTS: Among the 45 patients, 42 were females and the mean age at diagnosis was (50.8 +/- 8.1) years. The mean time interval between the first visit to physicians to the time of correct diagnosis was about 2 years. The most frequent symptoms were fatigue (66.7%, 30/45), then jaundice (55.6%, 25/45) and pruritus (40%, 18/45). Nine patients (20%) were asymptomatic. Nine patients (20%) were associated with other auto-immune diseases (Sjogren's syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (gamma-GT) levels were markedly elevated in all the patients [(498.5 +/- 369.8) IU/L and (595.2 +/- 518.4) IU/L, respectively], whereas ALT and AST levels were mildly to moderately elevated [(83.9 +/- 58.8) IU/L and (100.8 +/- 48.8) IU/L, respectively]. 25 patients (55.6%) had a total bilirubin level >/= 34.2 micromol/L. 40 patients (88.9%) had elevated serum IgM and 43 patients (95.6%) were anti-mitochondrial antibody (AMA)/AMA-M2 positive. CONCLUSION: In China, PBC is probably not so rare as it was thought before. It is mostly found in middle-aged women. The most frequent symptom is fatigue and some patients are asymptomatic at early stage. Elevated serum ALP and gamma-GT levels together with positive AMA/AMA-M2 can help to diagnose PBC. Liver biopsy is useful to confirm the diagnosis and differentiate histopathological stages.