Primary biliary cirrhosis:an analysis of 50 cases

Objective Clinical features of primary biliary cirrhosis(PBC) were reviewed in order to improve its diagnosis in our clinical practice.Methods Clinical data of 50 patients with PBC were reviewed including the clinical manifestation,laboratory tests,pathological findings and the response to therapy.Results Fifty patients were females(80%),10 patients were males(20%),and the mean age at diagnosis was 55.9±13.2 years.The most frequently complained symptoms were jaundice(62%),fatigue(54%),anorexia(46%) and pruitus(20%).Serum alkaline phosphatase(ALP) and glutamyl transpeptidase(GGT) levels were remarkably elevated in majority patients(239.4±140.1 U/L and 252.6±226.1 U/L,respectively),whereas ALT and AST levels were mildly to moderately elevated(196.8±285.2 U/L and 168.7±173.3 U/L,respectively).Twenty six patients(68%) had a total bilirubin level ≥34.2 μmol/L.Twenty patients(48.7%) had elevated serum IgM and 34 patients(68%) were anti-mitochondrial antibody(AMA)/AMA-M2 positive.Thirty patients underwent pathological examinations,16.7% in early stage(Ⅰ,Ⅱstage) and 83.3% in advanced stage(Ⅲ,Ⅳstage).Conclusions PBC most frequently affects middle-aged women and the main clinical manifestations are jaundice and some patients are asymptomatic at early stage.Elevated serum ALP and γ-GT levels together with positive AMA/AMA-M2 can help to diagnose.Liver biopsy is useful to confirm the diagnosis and differentiate histopathological stages.