Antimitochondrial Antibodies: from Bench to Bedside.

IF 8.4 2区 医学 Q1 ALLERGY Clinical Reviews in Allergy & Immunology Pub Date : 2022-10-01 DOI:10.1007/s12016-021-08904-y
Francesca Colapietro, Ana Lleo, Elena Generali
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引用次数: 25

Abstract

Anti-mitochondrial antibodies (AMA) are directed against the E2 subunits of the 2-oxo acid dehydrogenase complexes (PDC-E2) and are the typical biomarkers of primary biliary cholangitis (PBC), being present in 90-95% of patients, with increasing sensitivity at increasing titers. Albeit being highly specific for PBC diagnosis, AMA can be detected in less than 1% of healthy subjects, and thus the management subjects with no sign or symptom of liver disease is still a challenge and data concerning clinical risk of developing PBC in this subgroup of patients are controversial. Moreover, AMA can also be detected in patients affected by overlap syndrome, as well as hepatic diseases (i.e., NASH and viral hepatitis), while the association with autoimmune diseases, in particular Sjögren's syndrome, systemic sclerosis, and systemic lupus erythematosus, is well established. Furthermore, new associations are being identified with inflammatory myositis and heart disease. AMA are directed towards the pyruvate dehydrogenase multi enzyme complex (PDC-E2) subunit, which represents an epithelial specific autoantigen for PBC. This review focuses on the main characteristics of AMA, their association with autoimmune diseases and liver diseases.

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抗线粒体抗体:从实验室到临床。
抗线粒体抗体(AMA)针对2-氧酸脱氢酶复合物(PDC-E2)的E2亚基,是原发性胆道性胆管炎(PBC)的典型生物标志物,存在于90-95%的患者中,随着滴度的增加,其敏感性也随之增加。尽管AMA对PBC的诊断具有高度特异性,但在不到1%的健康受试者中可以检测到,因此没有肝脏疾病体征或症状的管理受试者仍然是一个挑战,有关该亚组患者发生PBC的临床风险的数据存在争议。此外,AMA也可在重叠综合征以及肝脏疾病(即NASH和病毒性肝炎)患者中检测到,而与自身免疫性疾病,特别是Sjögren综合征、系统性硬化症和系统性红斑狼疮的关联已得到证实。此外,炎症性肌炎和心脏病也有新的关联。AMA针对丙酮酸脱氢酶多酶复合物(PDC-E2)亚基,该亚基代表PBC的上皮特异性自身抗原。本文综述了AMA的主要特征及其与自身免疫性疾病和肝脏疾病的关系。
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来源期刊
CiteScore
22.30
自引率
1.10%
发文量
58
审稿时长
6-12 weeks
期刊介绍: Clinical Reviews in Allergy & Immunology is a scholarly journal that focuses on the advancement of clinical management in allergic and immunologic diseases. The journal publishes both scholarly reviews and experimental papers that address the current state of managing these diseases, placing new data into perspective. Each issue of the journal is dedicated to a specific theme of critical importance to allergists and immunologists, aiming to provide a comprehensive understanding of the subject matter for a wide readership. The journal is particularly helpful in explaining how novel data impacts clinical management, along with advancements such as standardized protocols for allergy skin testing and challenge procedures, as well as improved understanding of cell biology. Ultimately, the journal aims to contribute to the improvement of care and management for patients with immune-mediated diseases.
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