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.

Perspectives on a combined test of multi serum biomarkers in China: Towards screening for and diagnosing hepatocellular carcinoma at an earlier stage

“…Evidence has shown that surgical resection and liver transplantation may offer the best opportunity for treating HCC yet are only available to early-detected patients (13)(14)(15)(16). The normal overall 5-year survival rate is 40%, but with a liver resection to treat early HCC, the 5-year survival rate rise to 60-70% (17,18).…”

Peer Review #2 of "The long-term outcomes of deceased-donor liver transplantation for primary biliary cirrhosis: a two-center study in China (v0.1)"

“…Manuscript to be reviewed performed liver transplantaion for PBC is uncertain (Akamatsu & Sugawara 2012) , whether patients can benefit from protocal biopsies needs further study. Previous studies have identified several factors associated with rPBC, including age, number of HLA mismatches, and the use of immunosuppressants.…”

Association between the Globe Score, Uk-Score and The Response to Ursodesoxycholic Acid During Primary Biliary Cholangitis

“…To date, more than 6,000 liver transplants have been performed in the United States and Europe for PBC [31]. The number of liver transplants for PBC has decreased over the past two decades [17].…”