title: Primary biliary cirrhosis and liver transplantation.
abstract: Primary biliary cirrhosis (PBC) is an immune-mediated chronic progressive inflammatory liver disease, predominantly affecting middle-aged women, characterized by the presence of antimitochondrial antibodies (AMAs), which can lead to liver failure. Genetic contributions, environmental factors including chemical and infectious xenobiotics, autoimmunity and loss of tolerance have been aggressively investigated in the pathogenesis of PBC, however, the actual impact of these factors is still controversial. Survival of PBC patients has been largely improved with the widespread use of ursodeoxycholic acid (UDCA), however, one third of patients still do not respond to the treatment and proceed to liver cirrhosis, requiring liver transplantation as a last resort for cure. The outcome of liver transplantation is excellent with 5- and 10-year survival rates around 80% and 70%, respectively, while along with long survival, the recurrence of the disease has become an important outcome after liver transplantation. Prevalence rates of recurrent PBC rage widely between 1% and 35%, and seem to increase with longer follow-up. Center-specific issues, especially the use of protocol biopsy, affect the variety of incidence, yet, recurrence itself does not affect patient and graft survival at present, and retransplantation due to recurrent disease is extremely rare. With a longer follow-up, recurrent disease could have an impact on patient and graft survival.
authors.affiliation: Department of Hepato-biliary-pancreatic Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan; ; Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan.
authors.forename: Nobuhisa
authors.initials: N
authors.lastname: Akamatsu
authors.affiliation: Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, the University of Tokyo, Tokyo, Japan.
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Sugawara
keywords: Primary biliary cirrhosis; liver transplantation; ursodeoxycholic acid