Published online Dec 18, 2015. doi: 10.4254/wjh.v7.i29.2896
Peer-review started: July 22, 2015
First decision: August 25, 2015
Revised: November 7, 2015
Accepted: December 1, 2015
Article in press: December 2, 2015
Published online: December 18, 2015
Liver transplantation (LT) is the most effective treatment modality for end stage liver disease caused by many etiologies including autoimmune processes. That said, the need for transplantation for autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC), but not for primary sclerosing cholangitis (PSC), has decreased over the years due to the availability of effective medical treatment. Autoimmune liver diseases have superior transplant outcomes than those of other etiologies. While AIH and PBC can recur after LT, recurrence is of limited clinical significance in most, but not all cases. Recurrent PSC, however, often progresses over years to a stage requiring re-transplantation. The exact incidence and the predisposing factors of disease recurrence remain debated. Better understanding of the pathogenesis and the risk factors of recurrent autoimmune liver diseases is required to develop preventive measures. In this review, we discuss the current knowledge of incidence, diagnosis, risk factors, clinical course, and treatment of recurrent autoimmune liver disease (AIH, PBC, PSC) following LT.
Core tip: There is compelling evidence that autoimmune liver disease recur after liver transplantation, with incidence rates ranging from 10% to 50%. Recurrent autoimmune hepatitis and primary biliary cirrhosis do rarely impair patient and graft survival, but may require changing the immunosuppressive regimen, using corticosteroids or adding ursodeoxycholic acid, respectively. In a proportion of patients, recurrent primary sclerosing cholangitis progresses over years to a stage requiring re-transplantation.