Objective To study the clinical features of patients with primary biliary cirrhosis (PBC) in order to improve the doctors’ awareness of the disease. Methods General status, clinical manifestations and laboratory findings of 40 patients with PBC were reviewed. Thirty-seven patients were females (37/40), and the mean age at diagnosis was 50.5±7.8 years. The time interval from initial symptoms or preliminary diagnosis to final diagnosis was 24.0±23.6 months. Results The most frequently reported symptoms were fatigue (67.5%, 27/40), jaundice (60%, 24/40) and pruritus (32.5%, 17/40). Eight patients (20%) had associated auto-immune diseases (Sjogren’s syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and gamma glutamyl transpeptidase (γ-GT) levels were markedly elevated (520.3±382.3 IU/L and 648.6±529.1 IU/L, respectively) in all patients, while alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were mildly elevated (82.6±54.5 IU/L and 100.7±47.2 IU/L, respectively). Twenty-four patients (60%) had a total bilirubin level ≥34.2 μmol/L. Thirty-five patients (87.5%) had elevated serum immunoglobin M, and 97.5% of patients (39/40) were anti-mitochondrial antibody (AMA)/AMA-M2 positive. Conclusion Elevated serum ALP and γ-GT levels, together with a positive AMA/AMA-M2, can help the diagnosis of PBC. Liver biopsy is useful to confirm the diagnosis and to differentiate histopathological stages.
