Recurrence of autoimmune liver disease and inflammatory bowel disease after pediatric liver transplantation.
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.
Institute of Liver Studies and Transplantation, King's College London School of Medicine, King's College Hospital, London, UK.|Department of Gastroenterology, Centro Hospitalar São João, Faculty of Medicine, University of Porto, Porto, Portugal.
Rodrigo
R
Liberal
Institute of Liver Studies and Transplantation, King's College London School of Medicine, King's College Hospital, London, UK.
Diego
D
Vergani
Institute of Liver Studies and Transplantation, King's College London School of Medicine, King's College Hospital, London, UK.|Paediatric Liver, GI and Nutrition Centre, King's College Hospital, London, UK.
Giorgina
G
Mieli-Vergani
D002648:Child; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D058625:End Stage Liver Disease / Q000209:etiology / Q000601:surgery*; D006084:Graft Rejection / Q000517:prevention & control*; D006085:Graft Survival / Q000276:immunology; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000453:epidemiology / Q000276:immunology / Q000473:pathology; D008099:Liver / Q000276:immunology / Q000473:pathology; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D015995:Prevalence; D012008:Recurrence; D012698:Serologic Tests
D007166:Immunosuppressive Agents