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肝移植后新发自身免疫性肝炎。

De novo autoimmune hepatitis after liver transplantation.

机构信息

Department of Medical Sciences and Special Therapies--Pathology Unit, University of Padova, Padova, Italy.

出版信息

Semin Liver Dis. 2011 Feb;31(1):71-81. doi: 10.1055/s-0031-1272834. Epub 2011 Feb 22.

Abstract

Allograft dysfunction with clinical, serologic, and histologic features resembling autoimmune hepatitis (AIH) may develop in pediatric and adult patients who have received a liver transplant (LT) for end-stage diseases other than AIH. This condition is now known as de novo AIH, although its pathophysiology is still uncertain and whether it represents a specific type of rejection or a genuine form of AIH is under debate. The occurrence of de novo AIH seems to be unrelated to the etiology of the disease necessitating liver transplantation, but it has been correlated with antiviral treatment in cases of hepatitis C virus (HCV) infection recurring after LT. Several investigators have reported adverse outcome of de novo AIH, including graft failure, particularly in cases with late diagnosis. Prompt treatment with prednisone, with or without azathioprine (in addition to the basic immunosuppressive regimen), seems to be the best option. The histology of de novo AIH is characterized by an infiltrate rich in plasma cells with significant interface hepatitis and perivenular necro-inflammatory activity. These features are not specific for autoimmune damage; therefore, other causes of graft dysfunction must be excluded. The final diagnosis may be a challenge in patients with recurrent hepatitis C, and requires careful clinical and pathologic assessment.

摘要

同种异体移植物功能障碍伴临床、血清学和组织学特征类似于自身免疫性肝炎(AIH),可能发生于接受肝移植(LT)治疗非 AIH 终末期疾病的儿科和成年患者中。这种情况现在被称为新诊断 AIH,尽管其病理生理学仍不确定,并且它是否代表一种特定类型的排斥反应或真正的 AIH 形式仍存在争议。新诊断 AIH 的发生似乎与需要进行 LT 的疾病的病因无关,但与 LT 后丙型肝炎病毒(HCV)感染复发时的抗病毒治疗有关。一些研究人员报告了新诊断 AIH 的不良预后,包括移植物失功,特别是在诊断较晚的情况下。及时用泼尼松治疗,联合或不联合硫唑嘌呤(除了基本的免疫抑制方案外)似乎是最佳选择。新诊断 AIH 的组织学特征为富含浆细胞的浸润,具有显著的界面肝炎和门静脉周围坏死性炎症活动。这些特征不是自身免疫损伤的特异性;因此,必须排除移植物功能障碍的其他原因。在丙型肝炎复发的患者中,最终诊断可能具有挑战性,需要仔细的临床和病理评估。

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