Recurrence of autoimmune liver disease and inflammatory bowel disease after pediatric liver transplantation

Abstract

Approximately 10% of children with autoimmune hepatitis (AIH) and 30% of those with sclerosing cholangitis (SC) require liver transplantation (LT). LT is indicated in patients who present with fulminant hepatic failure (ie, with encephalopathy) and in those who develop end-stage liver disease despite treatment. After LT, recurrent AIH is reported in approximately 30% of patients and recurrent SC in up to 50%. Diagnosis of recurrence is based on biochemical abnormalities, seropositivity for autoantibodies, interface hepatitis on histology, steroid dependence, and, for SC, presence of cholangiopathy. Recurrence of SC after LT is often associated with poorly controlled inflammatory bowel disease (IBD). Recurrence may even appear years after LT; therefore, steroid-based immunosuppression should be maintained at a higher dose than that used for patients transplanted for nonautoimmune liver diseases. Although the impact of recurrent disease on graft function is controversial, it seems that in pediatric LT recipients recurrence of AIH or SC is associated with compromised graft survival. Exacerbation of preexistent IBD may be observed after LT for SC or AIH, and IBD appears to have a more aggressive course than before LT. In addition, IBD can develop de novo following LT. Liver Transplantation 22 1275-1283 2016 AASLD.

Systematic review: recurrent autoimmune liver diseases after liver transplantation

“…Exacerbation of pre‐existent IBD may be observed after LT for PSC, and the course of IBD appears to be more aggressive in comparison to pre‐LT disease …”

Autoimmune hepatitis: From current knowledge and clinical practice to future research agenda

“…44 Paediatric AIH may also be associated with intestinal inflammation either before or after liver transplantation, suggesting the importance of exploring the pathogenic concepts that involve the gut-liver axis in AIH. 45 This association is particularly observed in ASC, which may be an early or paediatric form of PSC, explaining the close relationship with intestinal inflammatory disease.…”

“…Patients who have biliary changes in addition to portal inflammation should be closely followed as up to 50% of patients presenting with AIH may have autoimmune sclerosing cholangitis (ASC) or an early form of PSC . Paediatric AIH may also be associated with intestinal inflammation either before or after liver transplantation, suggesting the importance of exploring the pathogenic concepts that involve the gut‐liver axis in AIH . This association is particularly observed in ASC, which may be an early or paediatric form of PSC, explaining the close relationship with intestinal inflammatory disease.…”

Autoimmune Hepatitis in Children

“…Otoimmun hepatitte karaciğer nakli sonrası 5 yıllık sağkalım oranı ise %80-90 olarak bildirilmektedir (1) . Fakat çocuklarda OİH ve OSK'de karaciğer nakli sonrası yaklaşık %30 oranında OİH ve %10-50 oranında OSK yineleyebilmektedir (23) .…”