A Review on Biomarkers for the Evaluation of Autoimmune Cholestatic Liver Diseases and Their Overlap Syndromes.

Frontiers in molecular medicine Pub Date : 2022-07-08 eCollection Date: 2022-01-01 DOI:10.3389/fmmed.2022.914505
Henry H Nguyen, Marvin J Fritzler, Mark G Swain
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Abstract

Autoimmune cholestatic liver disease includes both Primary Biliary Cholangitis (PBC) and Primary Sclerosing Cholangitis (PSC). Both conditions result in impairment of hepatic bile flow ultimately leading to chronic liver injury, liver fibrosis and eventually end stage cirrhosis. Early and accurate diagnosis are important for the risk stratification, follow up and management of these patients. The underlying pathogenesis of these conditions have not been completely resolved and poses a barrier for the development of new diagnostic and prognostics tools. Current research work suggests that the pathogenesis of autoimmune cholestatic liver disease results from environmental, genetic, and a large component of underlying immune dysfunction. While the current available serum biomarkers and imaging modalities showcases progression in precision medicine for the management of autoimmune cholestatic liver disease, development of new biomarkers are still an area of need in this field. In this review, we will discuss the current and emerging biomarkers in patients with PBC, PSC, and a special population that exhibit overlap syndrome with autoimmune hepatitis (AIH). The use of these biomarkers for diagnosis and prognosis of these patients will be reviewed through the lens of the current understanding of the complex immune pathophysiology of these conditions.

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评价自身免疫性胆汁淤积性肝病及其重叠综合征的生物标志物研究进展
自身免疫性胆汁淤积性肝病包括原发性胆汁性胆管炎(PBC)和原发性硬化性胆管炎(PSC)。这两种情况都会导致肝胆汁流受损,最终导致慢性肝损伤、肝纤维化,并最终导致终末期肝硬化。早期准确的诊断对这些患者的风险分层、随访和管理至关重要。这些疾病的潜在发病机制尚未完全解决,这对开发新的诊断和预测工具构成了障碍。目前的研究工作表明,自身免疫性胆汁淤积性肝病的发病机制是由环境、遗传和潜在免疫功能障碍的重要组成部分引起的。虽然目前可用的血清生物标志物和成像模式显示了精确医学在治疗自身免疫性胆汁淤积性肝病方面的进展,但开发新的生物标志物仍然是该领域需要的领域。在这篇综述中,我们将讨论PBC、PSC患者以及与自身免疫性肝炎(AIH)表现出重叠综合征的特殊人群中当前和新出现的生物标志物。将通过目前对这些疾病复杂免疫病理生理学的理解来回顾这些生物标志物在这些患者的诊断和预后中的应用。
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