Risk Factors for Survival and the Development of Hepatocellular Carcinoma in Patients with Primary Biliary Cirrhosis

Objective Early diagnosis of hepatocellular carcinoma (HCC) is critical in the management of patients with primary biliary cirrhosis (PBC), since the prognosis of PBC has improved. The aim of this study was to investigate whether HCC development affects the prognosis of PBC and to identify the risk factors for HCC in Japanese patients with PBC. Methods We compared the survival rates between patients with HCC and those without and analyzed the risk factors for HCC development in 210 patients with PBC who were followed up for a median period of 8.5 years. Results HCC developed during follow-up in 11 patients (5.2%) and was diagnosed simultaneously at the time of diagnosis of PBC in five patients (2.4%) who were excluded from the analysis. A Kaplan-Meier analysis showed a significant difference in overall survival between the patients who did and did not develop HCC (p<0.001). A multivariate analysis revealed age (OR: 1.08, 95% confidence interval [CI]: 1.03-1.13, p=0.001), the albumin level (OR: 0.24, 95% CI: 0.10-0.56, p=0.001), the total bilirubin level (OR: 1.60, 95% CI: 1.09-2.36, p=0.017) and HCC development (OR: 2.97, 95% CI: 1.24-7.15, p=0.015) to be significant prognostic factors and identified only an advanced histological stage (Scheuer's classification III or IV, OR: 6.27, 95% CI: 1.80-21.83, p=0.004) to be a risk factor associated with HCC. Conclusion HCC development significantly affects the survival of patients with PBC, and an advanced histological stage is the only risk factor associated with HCC development. These results highlight the important role of liver fibrosis in hepatocarcinogenesis in patients with PBC.