СОВРЕМЕННЫЕ ВОЗМОЖНОСТИ ДИГНОСТИКИ И ЛЕЧЕНИЯ СИСТЕМНЫХ ВАСКУЛИТОВ С ПОРАЖЕНИЕМ ПОЧЕК У ДЕТЕЙ: СОБСТВЕННЫЕ ДАННЫЕ (ЧАСТЬ 2)

Abstract

The aim of the study was to analyze clinical, laboratory, immunological, morphological data of children with systemic vasculitis (SV) with kidney involvement to assess mediators of the formation and progression of the disease, as well as factors affecting prognosis. Methods. 20 children (15 girls, 5 boys) with morphologically verified nephritis due to SV (age from 5 to 17 yrs, median 13.5), were under observation at the "2nd Children's Clinical Hospital" Minsk from 2012 -2020. The features of the onset of the disease in childhood are presented, the analysis of clinical, laboratory, immunological (serum concentration of T- and B-lymphocyte activation markers (RANTES and BAFF), proinflammatory (caspase 1, IL1β and TNFα), vascular (VEGF) and tissue (TGF1β) growth factors), metabolic parameters (adiponectin, leptin, obestatin, vitamin D 25 (OH) D), instrumental, morphological changes, and factors affecting the course and outcome. Statistics was carried out using the Statistica 10.0, SPSS. Results. A significant increase in the concentration of markers of activation of T and B lymphocytes (RANTES, BAFF), proinflammatory (caspase 1, TNFα, IL1β) and markers of endothelial dysfunction (VEGF and TGF1β) in children with nephritis due to SV in comparison with healthy children, as well as a decrease in the above mentioned molecules during remission, allows them to be used as markers of disease activity. High frequency of arterial hypertension during the period of active manifestations of the disease (85%) and on the multicomponent hypotensive therapy (70%), presence of nocturnal hypertension and/or insufficient nighttime blood pressure reduction in all patients, signs of cardiac remodeling (40%), pro-atherogenic metabolic disorders make it possible to classify patients with nephritis in SV as a high-risk group of early development of cardiovascular disorders, which requires careful monitoring and correction. Conclusion. SV in childhood is associated with severe organ damage and mortality (5%). Nephritis significantly adverse outcome of the disease, impaired renal function at the onset of the SV is noted in 65%, progression to end stage chronic kidney disease in 35% of cases. Recurrent course, persistent nephrotic proteinuria, serum creatinine, VEGF, adyponectin and obestatin, decrease in estimated glomerular filtration rate at the onset of the disease is associated with progression of nephritis in SV.