Clinical features of 70 patients with primary biliary cirrhosis

Objective Clinical features of primary biliary cirrhosis (PBC) were reviewed in order to improve its diagnosis in our clinical practice. Methods Clinical data of 70 patients with PBC were reviewed including the clinical manifestation, laboratory tests, pathological findings and the response to therapy. Results Sixty-two patients were females (88.6%), eight patients were males (11.4%), and the mean age at diagnosis was (53.1±10.4) years. The most frequently complained symptoms were pruitus (60.0%), fatigue (42.9%), anorexia (41.4%) and jaundice (45.7%). Serum alkaline phosphatase (ALP) and γ glutamyl transpeptidase (GGT) levels were markedly elevated in all patients, while alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were mildly elevated. The serum total bilirubin level increased in sixty-five patients (92.9%). Forty-four patients (62.9%) had elevated serum immunoglobin M (IgM), and 98.6% of patients (69/70) were antimitochondrial antibody (AMA) /AMA-M2 positive. Forty-one patients underwent pathological examinations, 82.9% in early stage (Ⅰ,Ⅱ stage) and 17.1% in advanced stage (Ⅲ,Ⅳ stage).Conclusions PBC most frequently affects middle-aged women and the main clinical manifestations are pruritus, fatigue, anorexia and jaundice. The elevated level of ALP, GGT, IgM and positive AMA/AMA-M2 may be crucial to diagnosis of PBC.