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Review
. 2022 Oct;63(2):166-177.
doi: 10.1007/s12016-021-08904-y. Epub 2021 Sep 29.

Antimitochondrial Antibodies: from Bench to Bedside

Francesca Colapietro  1   2 , Ana Lleo  3   4 , Elena Generali  1   2
Affiliations
Review

Antimitochondrial Antibodies: from Bench to Bedside

Francesca Colapietro et al. Clin Rev Allergy Immunol. 2022 Oct.

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.

Keywords: Autoimmunity; Myositis; Primary biliary cholangitis; Systemic sclerosis.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Anti-mitochondrial antibodies detection by indirect immunofluorescence using HEp-2 cell or mouse tissue sections. A AMA staining of mouse kidney/smooth muscle/stomach tissue section showing staining of both proximal and distal tubules of the mouse kidney (upper right) and the parietal cells of the mouse stomach (lower left). B AMA on HEp-2 cells
Fig. 2
Fig. 2
Proposed pathogenetic mechanisms of primary biliary cholangitis APC, antigen presenting cell; PDC-E2, pyruvate dehydrogenase complex; IL, interleukin; IFN, interferon; AMA, anti-mitochondrial antibodies; BEC, biliary epithelial cells
See this image and copyright information in PMC

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