预后模型与自身免疫性肝病

IF 3.2 3区 医学 Q2 GASTROENTEROLOGY & HEPATOLOGY
Daphne D'Amato , Marco Carbone
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引用次数: 0

摘要

自身免疫性肝病(AILDs)是一种病因不明、免疫介导的复杂疾病。在原发性胆道胆管炎(PBC)和自身免疫性肝炎(AIH)中,疾病调节药物可改善患者的生活质量和数量。原发性硬化性胆管炎(PSC)没有药物治疗,唯一接受的治疗是肝移植(LT)。PBC, PSC和AIH具有描述每种疾病患者原型的特征。另一方面,经典的疾病是不均匀的,每一个诊断的患者可能表现出一系列的临床、生化、血清学和组织学结果。奇怪的是,它们被认为是罕见疾病,但在欧洲和美国,它们合计约占LTs的20% %。这些患者的管理是复杂的,因为aild在临床实践中相对罕见,在发展专业知识方面存在挑战,疾病表现可能是偷偷摸摸的,临床表型和病程是异质的。预后模型是临床医生评估患者风险和为患者提供个性化护理的关键工具。本综述的目的是讨论aild管理的挑战以及可用的预后模型如何提供帮助。我们将讨论在aild中开发的预后模型,特别关注可以支持aild患者临床管理的预后模型:基于熊去氧胆酸(UDCA)反应和肝纤维化标志物的PBC模型;PSC中包括生物化学、疾病分期和放射学半定量标记;最后在AIH中,是疾病阶段和疾病活动的标志。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Prognostic models and autoimmune liver diseases

Autoimmune liver diseases (AILDs) are complex diseases with unknown causes and immune-mediated pathophysiology. In primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) disease modifying drugs are available which improve patient quality and quantity of life. In primary sclerosing cholangitis (PSC) no medical therapy is available and the only accepted treatment is liver transplantation (LT). PBC, PSC and AIH possess features that describe the archetype of patients within each disorder. On the other hand, the classical disorders are not homogeneous, and patients within each diagnosis may present with a range of clinical, biochemical, serological, and histological findings.

Singularly, they are considered rare diseases, but together, they account for approximately 20% of LTs in Europe and USA. Management of these patients is complex, as AILDs are relatively uncommon in clinical practice with challenges in developing expertise, disease presentation can be sneaky, clinical phenotypes and disease course are heterogeneous. Prognostic models are key tools for clinicians to assess patients’ risk and to provide personalized care to patients. Aim of this review is to discuss challenges of the management of AILDs and how the available prognostic models can help. We will discuss the prognostic models developed in AILDs, with a special focus on the prognostic models that can support the clinical management of patients with AILDs: in PBC models based on ursodeoxycholic acid (UDCA) response and markers of liver fibrosis; in PSC several markers including biochemistry, disease stage and radiological semiquantitative markers; and finally in AIH, markers of disease stage and disease activity.

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来源期刊
CiteScore
5.50
自引率
0.00%
发文量
23
审稿时长
69 days
期刊介绍: Each topic-based issue of Best Practice & Research Clinical Gastroenterology will provide a comprehensive review of current clinical practice and thinking within the specialty of gastroenterology.
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