PBC-transplantation and disease recurrence

Abstract

Despite near universal use of ursodeoxycholic acid (UDCA) several patients with PBC still progress to liver transplant (LT) or death. Pruritus and fatigue are the most common symptoms. Liver transplantation for pruritus is highly effective but fatigue will not disappear in the majority of the patients. In contrast to other liver diseases, portal hypertension may develop in pre-cirrhotic patients with PBC. Patients with PBC have an incidence rate of 3.4 hepatocellular carcinoma cases for every 1000 patient-years and risk factors are advanced stage of the disease and male sex. For the appropriate timing of LT the utility of prognostic models (bilirubin, Mayo risk score and MELD, in particular) and standard exception points in case of HCC are established. However, recent data from different part of the world demonstrated that PBC patients compared to patients with PSC have higher waiting-list mortality. Hyperlipidemia can be present in up to 80% of the patients but there is no evidence for an elevated cardiovascular risk, certainly not in relationship with LT. Patients transplanted for PBC suffer more frequently from acute cellular and also late cellular rejection. However, 5-year patient survival rates after LT of 80-85% is better than for most other indications. Recurrent PBC is reported in a range from 14% up to 42% after LT but in contrast to other autoimmune diseases graft loss due to recurrent disease is not a major issue. The type of immunosuppression after LT was found to be associated with the incidence of recurrence but since mediate-term impact on overall and graft survival is negligible, tacrolimus-based regimens remain standard at most centers. Observational studies suggest that long-term administration of UDCA following LT has a beneficial effect on recurrence of PBC. Therefore biomarkers after LT that may identify patients at risk for recurrence should be further explored to allows early medical intervention.

Keywords: Mortality liver transplantation; PBC recurrence; Primary biliary cholangitis; Pruritus; Transplantation rejection.