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自身免疫性肝炎:诊断与治疗概述

Autoimmune Hepatitis: A Diagnostic and Therapeutic Overview.

作者信息

Mercado Lydia A, Gil-Lopez Fernando, Chirila Razvan M, Harnois Denise M

机构信息

Department of Liver Transplant, Mayo Clinic Florida, Jacksonville, FL 32224, USA.

Department of General Internal Medicine, Mayo Clinic Florida, Jacksonville, FL 32224, USA.

出版信息

Diagnostics (Basel). 2024 Feb 9;14(4):382. doi: 10.3390/diagnostics14040382.

Abstract

Autoimmune hepatitis is an immune-mediated inflammatory condition of the liver of undetermined cause that affects both sexes, all ages, races, and ethnicities. Its clinical presentation can be very broad, from having an asymptomatic and silent course to presenting as acute hepatitis, cirrhosis, and acute liver failure potentially requiring liver transplantation. The diagnosis is based on histological abnormalities (interface hepatitis), characteristic clinical and laboratory findings (increased aspartate aminotransferase, alanine aminotransferase, and serum IgG concentration), and the presence of one or more characteristic autoantibodies. The large heterogeneity of these clinical, biochemical, and histological findings can sometimes make a timely and proper diagnosis a difficult task. Treatment seeks to achieve remission of the disease and prevent further progression of liver disease. First-line therapy includes high-dose corticosteroids, which are later tapered to decrease side effects, and azathioprine. In the presence of azathioprine intolerance or a poor response to the standard of care, second-line therapy needs to be considered, including mycophenolate mofetil. AIH remains a diagnostic and therapeutic challenge, and a further understanding of the pathophysiological pathways of the disease and the implementation of randomized controlled trials are needed.

摘要

自身免疫性肝炎是一种病因不明的免疫介导的肝脏炎症性疾病,可影响所有性别、年龄、种族和民族。其临床表现范围很广,从无症状和隐匿病程到表现为急性肝炎、肝硬化以及可能需要肝移植的急性肝衰竭。诊断基于组织学异常(界面性肝炎)、特征性的临床和实验室检查结果(天冬氨酸转氨酶、丙氨酸转氨酶和血清IgG浓度升高)以及一种或多种特征性自身抗体的存在。这些临床、生化和组织学检查结果的巨大异质性有时会使及时、准确的诊断成为一项艰巨任务。治疗旨在实现疾病缓解并防止肝病进一步进展。一线治疗包括高剂量皮质类固醇,随后逐渐减量以减少副作用,以及硫唑嘌呤。在存在硫唑嘌呤不耐受或对标准治疗反应不佳的情况下,需要考虑二线治疗,包括霉酚酸酯。自身免疫性肝炎仍然是一个诊断和治疗挑战,需要进一步了解该疾病的病理生理途径并开展随机对照试验。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/196b/10887775/dfa0f584dedb/diagnostics-14-00382-g0A1.jpg

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