De-novo autoimmune hepatitis after liver transplantation
De-novo autoimmune hepatitis after liver transplantation
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DOI：
10.1016/s0140-6736(97)06478-7
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发表时间：
1998-02-07
期刊：
影响因子：
168.9
通讯作者：
Mieli-Vergani, G
中科院分区：
文献类型：
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作者：
Kerkar, N;Hadzic, N;Mieli-Vergani, G
背景肝移植术后可能发生并非由公认原因引起的晚期移植物功能障碍，如排斥反应、感染或血管或胆道并发症。我们调查了1991年至1996年在我单位接受肝移植的儿童中与自身免疫特征相关的一种特殊类型的不明原因的移植物功能障碍。方法180例肝移植受者中有7例（4%）发生了一种不明原因但特征性的移植物功能障碍（5例男孩，2例女孩;中位年龄10.3岁，范围2.0-19.4岁）。术后中位时间为24个月（6-45）。肝外胆道闭锁（4例）、Alagille综合征（1例）、药物性急性肝功能衰竭（1例）和α 1-抗胰蛋白酶缺乏症（1例）是移植的适应症。4例患者接受环孢菌素、硫唑嘌呤和泼尼松龙三联免疫抑制治疗，3例接受他克莫司治疗，进行免疫球蛋白测定、自身抗体研究、血清学研究和HLA分型。肝活检samples.Findings感染和手术并发症肝活检标本显示慢性肝炎的组织学变化，包括门静脉和门静脉周围肝炎淋巴细胞和崩溃，和典型的急性或慢性排斥反应的小静脉周围细胞坏死。所有患者均具有高浓度IgG（中位数22 g/L [范围17.2-34.4]）和高滴度自身抗体。除一名患者外，所有患者均在中位32天内（7-316）对泼尼松龙2 mg/kg/d和硫唑嘌呤（1.5 mg/kg/d）的增加或添加有反应。一名反应者因依从性差而复发，但在恢复治疗后缓解。所有六个受访者保持在缓解减少剂量的泼尼松龙（5-10毫克/天）和1.5毫克/微克，每天硫唑嘌呤在中位数为283天（范围108-730）follow-up.Interpretation我们的数据显示，自身免疫性肝炎，这是响应于经典的治疗这种情况下，可以出现在肝移植患者，而他们是对抗排斥免疫抑制的症状。这些患者的肝损伤是一种排斥反应还是自身免疫性附着的结果尚未确定。
Background Late graft dysfunction that does not result from recognised causes, such as rejection, infection, or vascular or biliary complications, can occur after liver transplantation. We investigated a particular type of unexplained graft dysfunction that is associated with autoimmune features in children who underwent transplantation at our unit between 1991 and 1996.Methods Seven (4%) of 180 liver-transplant recipients developed an unexplained but characteristic form of graft dysfunction (five boys, two girls; median age at presentation 10.3 years, range 2.0-19.4). The median period after surgery was 24 months (6-45). The indications for transplantation had been extrahepatic biliary atresia (four patients), Alagille's syndrome (one), drug-induced acute liver failure (one), and alpha(1)-antitrypsin deficiency (one). Four patients were on triple immunosuppression with cyclosporin, azathioprine, and prednisolone; and three were on tacrolimus, Immunoglobulin measurements, autoantibody studies, serological studies, and HLA typing were undertaken. Liver-biopsy samples were taken.Findings Infectious and surgical complications were Liver-biopsy samples showed the histological changes of chronic hepatitis, including portal and periportal hepatitis with lymphocytes and collapse, and perivenular-cell necrosis typical of acute or chronic rejection. All patients had high concentrations of IgG (median 22 g/L [range 17.2-34.4]) and high titres of autoantibodies. All but one patient responded to prednisolone 2 mg/kg daily and an increase in or addition of azathioprine (1.5 mg/kg daily) within a median of 32 days (7-316), One responder relapsed owing to poor compliance but went into remission after treatment was restored. All six respondents remain in remission on a reduced dose of prednisolone (5-10 mg/day) and 1.5 mg/hg daily azathioprine at a median of 283 days (range 108-730) follow-up.Interpretation Our data show that symptoms of autoimmune hepatitis, which are responsive to the classical treatment for this condition, can appear in liver-transplant patients while they are on anti-rejection immunosuppression. Whether the liver damage in these patients is a form of rejection or the consequence of autoimmune attach has yet to be established.