Syniachenko O.V.1, Yehudina Ye.D.2, Mikuksts V.Ya.1, Iermolaieva M.V.1, Keting E.V. 1, Dyadyk O.O.3

Summary. Among rheumatological disease the profile of the share of systemic sclerosis (SSc) is about 2%, and the number of such patients is increasing everywhere. One of the main clinical manifestations of SSc is a vascular lesion, and angiopathy symptoms occur in the morphological study of the skin and internal organs in all cases. Variants of the course of vascular pathology in these patients are not studied well. The purpose and objectives. To evaluate the course of vascular lesions in patients with SSc according to the instrumental methods of diagnosis, the nature of the relationship with the morphological manifestations of skin vasculopathy and kidney, the role of the immune system disorders and vascular endothelial function in the pathogenesis of scleroderma angiopathy. Material and methods. The study included 57 patients with SSc (6 men and 51 women with an average age 42 years). The duration of the clinical manifestation of the disease was 11 years. A chronic clinical course of SSc has been in all surveyed, the I degree activity of the pathological process is ascertained in 40% of cases, II — 37%, III — 23%. Conducted clinical and ultrasound investigations (echocardiography, Doppler of blood vessels), biomicroscopy of vessels of the conjunctiva, in 37 patients — skin biopsy, and in 8 of them — renal biopsy. Results. The manifest vascular lesion occurs in 88% of patients with SSc, clinical, instrumental and morphological features of which are closely related to the degree of activity of pathological process and the duration of the disease, the nature of the «vegetative passport» (vagotonic and simpathotonic type of autonomic nervous system), with seropositivity of the diseases on anti-topoisomerase antibodies, antinuclear factor, antibodies to native deoxyribonucleic acid and cardiolipin. Angiopathy in SSc is accompanied by an increase of pulmonary vascular resistance, development of pulmonary hypertension and change processes of vasodilation, while the integrated vascular clinical, instrumental and morphological parameters have an influence on the severity of scleroderma pneumopathy and nephropathy, severity of endothelial cell proliferation, lymphohistiocytic infiltration and microthrombosis of vessels. Immunological parameters, along with an integral level of vascular endothelial dysfunction, participate in the pathogenetic constructions of vasculopathy in SSc and have prognostic significance in these patients.

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