OBJECTIVEIn order to improve the cognition and early diagnosis of primary biliary cirrhosis (PBC), we investigated clinical and pathological characteristics of PBC.

METHODSClinical data of 37 PBC patients together with pathological findings of 20 PBC patients were reviewed.

RESULTSAmong the 37 patients, 35 were women and the mean age at diagnosis was (53.4 8.9) years. The most frequent clinical presentations were jaundice (70.3%), fatigue (70.3%), and pruritus (56.8%). Serum glutamyl transpeptidase (GGT) and alkaline phosphatase (ALP) levels were markedly elevated in all patients (P50 was 467.50 U/L and 424.00 U/L, respectively). Among the 37 cases, 35 (94.6%) had total bile acids elevation, 32 (86.5%) had serum cholesterol elevation and 32 (86.5%) serum IgM elevation, 34 (91.9%) were positive for anti-mitochondrial antibody (AMA) and/or AMA-M2. Liver biopsy in 20 PBC patients mainly found: fibrosis in 17 cases (85%), interlobular bile duct lesions in 13 (65%), lobular mononuclear inflammation in 13 (65%), lymphocytic hepatocellular piecemeal necrosis in 10 (50%), and bile pigment accumulation in 9 (45%). The results of nonparametric test showed that GGT was related with pathological stage (P=0.002) and interlobular bile duct lesions (P=0.01).

CONCLUSIONSPBC is mostly found in middle-aged women. Accurate and prompt diagnosis of PBC should be based on the clinical presentation, biochemical and immunological indexes, and hepatic pathological changes. The level of GGT may partly reflect the severity of the histological lesions