Therapeutic Outcomes and Factors Affecting the Prognosis in Primary Biliary Cirrhosis

Introduction: The prognosis of primary biliary cirrhosis (PBC) is affected by its complication such as portal hypertension (PH) or hepatocellular carcinoma (HCC). In this study, efficacy of Ursodeoxycholic acid(UDCA) with or without Bezafibrate(BF) as well as factors associated with the prognosis of PBC was evaluated. Methods: Thirty-six patients (male/female 5/31, age at diagnosis 58.3±9.3, median observation period 114.5 months) diagnosed as PBC histologically or clinically during the period between 1994 and 2014 were enrolled. Liver-biopsy was conducted in 25 patients, consisting of 10/9/5/1 in Scheuer staging 1/2/3/4, respectively. Among these 36 patients, 23 received only UDCA, 12 received both UDCA and BF, and 1 received only BF. The trend of serum parameters (AST, ALT, ALP, gamma-GTP, T.Bil, PLT) after treatment as well as the relation between change in serum parameters (same as above) and incidence of complications (HCC, PH etc.) was assessed. Results: In all patients, each parameter such as AST, ALT, ALP and gamma-GTP (before treatment/3 months after treatment) was 76.3±93.7/36.9±20.8 IU/l, 80.4±89.7/32.2±19.9 IU/l, 706.1±365.5/443.8±196.1 IU/l, 271.9±215.2/89.0±67.9 IU/l, respectively, indicating significant amelioration (p150,000; group B: PLT < 150,000), the mortality rate after 6 months based on the predictive formula of prognosis by Japanese Liver Transplantation Society was higher in group B (P < 0.05). Furthermore, more complications were observed in group B (A/B: 11%(3/27)/44%(4/9)). Thus, the low number of PLT at diagnosis could be associated with higher incidence of complications. Conclusion: In patients with PBC, either UDCA monotherapy or combination therapy using UDCA and BF could be useful in ameliorating serum parameters. The state of symptoms as well as the number of PLT at diagnosis could be associated with the prognosis of PBC.