Journal of Pediatric Gastroenterology and Nutrition · 1997 · 97 citations · 14 references
MedicineImmunologyPediatricsAutoimmunityHepatologyTransplant SurgeryAutoimmune DiseasesCirrhosisLiver TransplantationPediatric HepatologyPediatric Liver DiseasePediatric TransplantationAutoimmune Hepatitis TreatmentAutoimmune Liver Disease TransplantationPediatric Autoimmune HepatitisPediatric Autoimmune Liver Disease TreatmentRecurrenceRecurrent Autoimmune Liver Disease
Liver transplantation is the standard treatment for end‑stage liver disease caused by chronic active autoimmune hepatitis, yet recurrence has been reported in up to 25 % of adult recipients. In a retrospective review of six pediatric patients who underwent orthotopic liver transplantation for autoimmune hepatitis, five experienced recurrence at a mean of 11.4 months post‑transplant. Recurrent autoimmune hepatitis was highly aggressive in children, with five of six patients relapsing within 11.4 months, three progressing to cirrhosis and retransplantation within a year, and all retransplanted patients experiencing a second recurrence; despite tacrolimus therapy, recurrence occurred in all four tacrolimus‑treated patients, indicating that transplantation is largely palliative and potent immunosuppression does not prevent relapse.
Liver transplantation is recognized as the appropriate treatment for end-stage liver disease due to chronic active autoimmune hepatitis. While it was initially thought that the disease did not recur after transplant, it is now generally accepted that adult patients may develop recurrent disease, with studies reporting a recurrence rate of < or = 25%. We have noted a higher incidence of recurrent autoimmune hepatitis in our pediatric patients undergoing liver transplant, with a high incidence of associated morbidity.We reviewed the records of six children followed up for autoimmune hepatitis who underwent orthotopic liver transplant for complications of end-stage liver disease.Of the six, five developed recurrent autoimmune hepatitis at a mean time of 11.4 months after transplant. The disease was aggressive, leading to cirrhosis and retransplant in three patients, within 1 year of recurrence. A second recurrence of disease occurred in all three retransplanted patients. One patient has received a third liver transplant, one has died, and one patient is asymptomatic on medical therapy. Autoimmune hepatitis recurred in all four patients receiving tacrolimus.We conclude that liver transplant for autoimmune hepatitis is likely to be palliative for most pediatric patients. Potent immunosuppressives such as tacrolimus do not protect against the development of recurrent autoimmune hepatitis.
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Liver Transplantation in European Patients with the Hepatitis B Surface Antigen
Didier Samuel, R. Müller, Graeme Alexander et al. · New England Journal of Medicine · 1993 · 1.1K citations · Full text
Dale C. Snover, Richard K. Sibley, Deborah K. Freese et al. · Hepatology · 1984 · 216 citations · Full text
Medicine, Pathology, Hepatology +16