The aim – to evaluate clinical course of cardiomyopathy in systemic vasculitis associated with antineutrophil antibodies (ANCA-SV), relation of heart disease and changes in large vessels to extracardiac manifestations of the disease, blood antibodies to myeloperoxidase and proteinase-3, vascular endothelial function and serum adsorption-rheological properties.
Material and methods. The study included 129 patients (47 % of men and 53 % of women) with ANCA-SV, among them 59 % with microscopic polyangiitis (MPA), 20 % with Wegener granulomatous polyangiitis (GPA) and 21 % with Churg – Strauss eosinophilic polyangiitis (EPA).
Results. Heart pathology signs were revealed in 62 % of patients with MPA, 50 % with GPA, 52 % with EPA. Among patients with MPA myocardial damage was diagnosed in 83 % cases, endocardium and valve apparatus – in 64 %, in GPA patients – in 92 % and 62 %, respectively, and in EPA – in 86 % and 64 %. Heart involvement was closely related to the pathology of joints, lungs, kidneys, liver and nervous system, determined by the severity of major vascular injury and their ability to vasodilation, depended on age of the patients, renal function and pulmonary artery pressure.
Conclusions. The pathology of the heart in ANCA-SV is one of the most frequent manifestations of disease associated with extracardiac signs of MPA, GPA and EPA.