Objective: The aim of this study was to describe the clinical characteristics of hepatitis C virus (HCV)-infected patients with primary biliary cirrhosis (PBC) by comparison to patients with both antimitochondrial (AMA) positive and AMA negative PBC.
Methods: All patients consecutively diagnosed as having PBC between 1973 and 1999 who had a regular follow-up of at least 2 yr were prospectively included in the study. The mean follow-up was 8.3 +/- 5.7 yr. Survival was calculated according to Kaplan-Meier curves.
Results: A total of 170 patients with PBC were considered. The syndrome with PBC and HCV infection (HCV-infected PBC patients) was recorded in 14 patients (13 women and one man), whereas 135 patients had AMA positive PBC and 18 had AMA negative PBC. Only three patients fulfilled the criteria for overlap syndrome involving PBC and autoimmune hepatitis. At presentation, the HCV-infected PBC group had significantly lower levels of ALP, gamma-glutamyl transpeptidase, and IgM than the AMA positive or AMA negative PBC patients (p < 0.01). With regard to the autoantibody profile, there was a significant association with LKM and HCV-infected PBC patients (21.4%), whereas ANA was significantly higher in AMA negative PBC patients than in the other two groups (83% vs 21.4% in the HCV-infected PBC patients and 38.5% in the AMA positive PBC group). No differences were found regarding the association with autoimmune conditions. During follow-up, hepatocellular carcinoma (HCC) developed more frequently in the PBC/HCV overlap group (i.e., three of 14 vs four of 135 patients with AMA positive PBC, p < 0.05). Survival curves were similar in HCV-infected PBC patients and AMA positive PBC, whereas the AMA negative group had a significantly slower decline (relative risk (RR) = 2.44, p < 0.05).
Conclusion: HCV-infected PBC patients are characterized by a biochemical profile with a modest rise in cholestatic enzymes but a high risk of developing HCC during follow-up.