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Published in: Clinical Reviews in Allergy & Immunology 2/2022

29-09-2021 | Systemic Lupus Erythematosus

Antimitochondrial Antibodies: from Bench to Bedside

Authors: Francesca Colapietro, Ana Lleo, Elena Generali

Published in: Clinical Reviews in Allergy & Immunology | Issue 2/2022

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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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Metadata
Title
Antimitochondrial Antibodies: from Bench to Bedside
Authors
Francesca Colapietro
Ana Lleo
Elena Generali
Publication date
29-09-2021
Publisher
Springer US
Published in
Clinical Reviews in Allergy & Immunology / Issue 2/2022
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-021-08904-y

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