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Autoimmune cardiomyopathy: a myth or reality?

Khashchevskaya D. A., Mitrofanova L. B., Ignatjeva E. S., Konovalov P. V., Malikov K. N., Lebedev D. S., Zverev D. A., Moiseeva O. M.
Federal State Budgetary Institution, “North-West Federal Medical Research Center” of the RF Ministry of Health Care, Akkuratova 2, St. Petersburg 97341

Keywords: autoantibody, autoimmune cardiomyopathy, myocarditis

DOI: 10.18087/rhfj.2015.5.2105

Background. Diversity of clinical manifestations of myocardial inflammatory disease and the need for morphological confirmation of diagnosis explain the difficulties which a physician encounters in examination and treatment of such patients. Aim. Comparative analysis of data from clinical and instrumental examination of patients with morphologically documented autoimmune cardiomyopathy (CMP) and lymphocytic myocarditis. Materials and methods. 11 patients with morphologically documented autoimmune CMP and 11 patients with active lymphocytic myocarditis and minimal HLA-DR expression were examined. Standard EchoCG study, contrast-enhanced magnetic resonance imaging, and endomyocardial biopsy (EMB) with immunogenic analysis were performed for all patients. Profile of circulating marker cardiotropic autoantibodies (autoAB) was determined, and biopsy samples were examined for cardiotropic viruses using molecular genetic methods. Results. Patients of two groups differed in the amount of myocardium-infiltrating CD3+-lymphocytes and pronouncement of the HLA-DR expression. 36 % of patients with documented myocarditis had cardiomyocyte necrosis associated with a higher detection frequency of the viral genome (64 %). The immunohistochemical analysis showed that the expression of myocardial immunoglobulins M (IgM) was observed only in patients with autoimmune CMP. Patients with autoimmune CMP had higher titers of autoAB to cytoskeletal and cytoplasmic proteins of cardiomyocytes and lower titers of autoAB to epitopes of adenine nucleotide translocator (ANT) than patients of the myocarditis group. Along with that, levels of circulating autoAB to β1-adrenoceptors and М2-cholinoreceptors were increased. According to data of contrast-enhanced MRI, a combination of two criterial signs for myocarditis (myocardial edema on T2‑weighted image and late contrast enhancement) was observed in 29 % of patients with autoimmune CMP and in 50 % of patients with myocarditis. Conclusion. It cannot be excluded that autoimmune CMP is only a specific case of the autoimmune inflammatory process in the myocardium, which could not be detected by EMB due to mosaicism of the process. The degree of myocardial inflammatory infiltration and the HLA-DR expression depend on the status of cell / humoral immunity and can be genetically determined.
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Khashchevskaya D. A., Mitrofanova L. B., Ignatjeva E. S., Konovalov P. V., Malikov K. N., Lebedev D. S. et al. Autoimmune cardiomyopathy: a myth or reality? Russian Heart Failure Journal. 2015;16 (5):288–295

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