TY  - JOUR
AU  - Dong, Victor
AU  - Montano-Loza, Aldo
AU  - Mason, Andrew
PY  - 2019
DA  - 2019/12/13
TI  - Modelling Recurrent Primary Biliary Cholangitis and Primary Sclerosing Cholangitis as Infectious Diseases Following Liver Transplantation
JO  - OBM Transplantation
SP  - 094
VL  - 03
IS  - 04
AB  - Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are idiopathic and progressive autoimmune hepatobiliary disorders that lead to liver failure and a need for liver transplantation in a proportion of individuals with poorly controlled disease.  It is currently thought that an environmental agent triggers disease in a genetically susceptible host and to date, xenobiotics, bacteria and a human betaretrovirus have all been linked with PBC. However, there is no consensus on which agents predominates. These disease processes are poorly understood and there are disparate hypotheses concerning the pathogenesis. One theory suggests that the disease is mediated by autoimmunity, whereas others have speculated that they are infectious disease processes that only manifests in individuals with diminished immunity. Clinically, the triggers of disease are difficult to study because of the indolent onset and chronic nature of the disorders. Notably, observations from liver transplantation provide a unique insight into the development of PBC and PSC. Both biliary disorders may reoccur in up to 30%-50% of patients following liver transplantation and many of the factors that influence recurrence have been well described. Prior to transplantation, immunosuppression is not routinely used to treat PBC and PSC because specific treatments have not been shown to have utility or have caused undue side effects. Following transplantation, recurrence occurs earlier and tends to be more aggressive in those treated with more potent immunosuppressive agent such as tacrolimus as compared to cyclosporine, which also has broad antiviral activity. The development of cholestasis within the first year following liver transplantation was found to be predictive of recurrence years later, a finding that parallels observations in patients with recurrent viral hepatitis following liver transplantation. Herein, we discuss the observations from liver transplant recipients with recurrent autoimmune liver disease and model our findings in comparison with patients that develop recurrent infectious disease. These studies help provide a framework and understanding of the processes associated with autoimmune liver diseases in general.
SN  - 2577-5820
UR  - https://doi.org/10.21926/obm.transplant.1904094
DO  - 10.21926/obm.transplant.1904094
ID  - Dong2019
ER  -