Birnbaum A H, Benkov K J, Pittman N S, McFarlane-Ferreira Y, Rosh J R, LeLeiko N S
Department of Pediatrics, Mount Sinai Medical Center, New York, New York, USA.
J Pediatr Gastroenterol Nutr. 1997 Jul;25(1):20-5. doi: 10.1097/00005176-199707000-00003.
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.
肝移植被认为是治疗慢性活动性自身免疫性肝炎所致终末期肝病的合适方法。虽然最初认为该疾病在移植后不会复发,但现在普遍认为成年患者可能会出现复发性疾病,研究报告的复发率≤25%。我们注意到在接受肝移植的儿科患者中,自身免疫性肝炎复发的发生率较高,且相关发病率也很高。
我们回顾了6名因终末期肝病并发症接受原位肝移植并随访自身免疫性肝炎的儿童的记录。
6名患者中,5名在移植后平均11.4个月出现复发性自身免疫性肝炎。该疾病进展迅速,3名患者在复发后1年内发展为肝硬化并再次移植。所有3名再次移植的患者均出现疾病二次复发。1名患者接受了第三次肝移植,1名患者死亡,1名患者在接受药物治疗后无症状。接受他克莫司治疗的所有4名患者均出现自身免疫性肝炎复发。
我们得出结论,对于大多数儿科患者而言,自身免疫性肝炎的肝移植可能只是姑息性治疗。像他克莫司这样强效的免疫抑制剂并不能预防复发性自身免疫性肝炎的发生。