自身免疫性肝炎的历史方面和当前认识。什么时候需要肝移植?(审查)

I. Iljinsky, O. Tsirulnikova
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摘要

自身免疫性肝炎(AIH)可以发生在任何年龄,在女性中更常见。这种疾病是暴露于某些环境因素的遗传易感人群的自身免疫易感性的表现。AIH的发病机制尚不完全清楚,但它涉及一种侵袭性的细胞免疫反应。AIH的发病机制和严重程度也取决于各种细胞因子。这种疾病的特点是转氨酶水平升高-天冬氨酸转氨酶(AST)和丙氨酸转氨酶(ALT)。肝脏组织学在确认或支持AIH的临床诊断中起着至关重要的作用。AIH的诊断在临床实践中仍然是一个挑战。AIH是为数不多的药物治疗已被证明可以提高生存率的肝脏疾病之一。标准治疗是单独使用大剂量强的松或强的松加硫唑嘌呤。它导致80%-90%的患者疾病缓解。大约20%的患者对标准类固醇治疗无效,并接受二线免疫抑制药物治疗:霉酚酸酯、布地奈德、环孢素、他克莫司、依维莫司和西罗莫司。有使用英夫利昔单抗和利妥昔单抗的报道。在AIH的自然病程和对治疗的抵抗中,有发展为肝硬化和疾病进展到终末期的趋势。这些患者,以及那些诊断为暴发性肝衰竭的患者,需要肝移植。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Historical aspects and current understanding of autoimmune hepatitis. When is liver transplantation indicated? (Review)
Autoimmune hepatitis (AIH) can occur at any age and is more common in women. The disease is a manifestation of autoimmune predisposition caused in genetically susceptible people exposed to certain environmental factors. The pathogenetic mechanism of AIH is not yet fully understood, but it involves an aggressive cellular immune response. The pathogenesis and severity of AIH also depend on various cytokines. This disease is characterized by elevated levels of transaminases – aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Liver histology plays a crucial role in confirming or supporting the clinical diagnosis of AIH. Diagnosis of AIH remains a challenge in clinical practice. AIH is one of the few liver diseases for which pharmacologic treatment has been shown to improve survival. Standard treatment is based on high-dose prednisone alone or prednisolone plus azathioprine. It leads to disease remission in 80%-90% of patients. Approximately 20% of patients do not respond to the standard steroid treatment and are treated with second-line immunosuppressive drugs: mycophenolate mofetil, budesonide, cyclosporine, tacrolimus, everolimus, and sirolimus. There have been reports on the use of infliximab and rituximab. In the natural course of AIH and resistance to therapy, there is a tendency for cirrhosis to develop and for the disease to progress to an end stage. These patients, as well as those diagnosed with fulminant liver failure, require liver transplantation.
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