Circulating Anti-GB3 Antibody as Biomarker of Myocardial Inflammation in Patients with Fabry Disease Cardiomyopathy

Andrea Frustaci; Romina Verardo; Michele Magnocavallo; Rossella Scialla; Luigi Sansone; Matteo Antonio Russo

doi:10.20944/preprints202304.0535.v1

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preprints.org > medicine and pharmacology > cardiac and cardiovascular systems > doi: 10.20944/preprints202304.0535.v1

Preprint Article Version 1 Preserved in Portico This version is not peer-reviewed

Circulating Anti-GB3 Antibody as Biomarker of Myocardial Inflammation in Patients with Fabry Disease Cardiomyopathy

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Version 1 : Received: 18 April 2023 / Approved: 19 April 2023 / Online: 19 April 2023 (04:01:57 CEST)

A peer-reviewed article of this Preprint also exists.

Frustaci, A.; Verardo, R.; Magnocavallo, M.; Scialla, R.; Sansone, L.; Russo, M.A. Circulating Anti-GB3 Antibody as a Biomarker of Myocardial Inflammation in Patients with Fabry Disease Cardiomyopathy. J. Clin. Med. 2023, 12, 4068. Frustaci, A.; Verardo, R.; Magnocavallo, M.; Scialla, R.; Sansone, L.; Russo, M.A. Circulating Anti-GB3 Antibody as a Biomarker of Myocardial Inflammation in Patients with Fabry Disease Cardiomyopathy. J. Clin. Med. 2023, 12, 4068.

Abstract

Background: Fabry disease cardiomyopathy (FDCM) has manifested some resistance to enzyme replacement therapy (ERT) particularly in its advanced phase. Recently myocardial inflammation of autoimmune origin has been demonstrated in FDCM. Aims: Aim of the study is the assessment of circulating anti-globotriaosylceramide (GB3) antibody as biomarker of myocardial inflammation in FDCM. Its sensitivity was based on the evidence of overlapping myocarditis at left ventricular endomyocardial biopsy. Methods and Results: From January 1996 to December 2021, 85 patients received in our Department the histological diagnosis of FDCM and 48 (56.5%) of them had an overlapping myocardial inflammation with negative PCR for the common cardiotropic viruses, positive anti-heart and anti-myosin abs. The presence of anti-GB3 antibodies was evaluated with an Elisa assay (BioGeM scarl Medical Investigational Research, MIR - Ariano Irpino, Italy) in the FDCM patients and compared with controls of healthy individuals. Circulating levels of anti-GB3 autoantibodies were compared with the presence or absence of myocardial inflammation at histology and with the severity of FDCM. The assay results showed that 42 out of 48 FDCM subjects with myocarditis were positive for anti-Gb3 antibodies while 30 out of 37 FDCM patients without myocarditis had negative anti-GB3 antibodies, with a sensitivity of 87.5 % and a specificity of 83.5%. Conclusions: The present study suggests a potential positive role of antiGB3 antibodies as marker of overlapping cardiac inflammation in patients with FDCM.

Keywords

Fabry Disease, Myocarditis, globotriaosylceramide, inflammation, cardiac biopsy.

Subject

Medicine and Pharmacology, Cardiac and Cardiovascular Systems

Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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