Treatment strategy ANCA-associated renal vasculitides in children and adolescents

Abstract

The article presents the terminology and classification in accordance with the International Сhapel Hill Сonsensus Сonference nomenclature of vasculitides (2012), clinical, immunological and morphological manifestations, therapy strategy and outcome of Antineutrophil cytoplasmic antibody vasculitides (ANCA) renal associated vasculitis (microscopic polyanghiitis, granulomatosis with Wegener's polyangiitis, eosinophilic granulomatosis with polyangiitis Churg-Strauss) in children and adolescents. IgG class antibodies to MPO and PR3, histopathological changes in renal biopsy specimens are considered the gold standard in the diagnosis of ANCA-glomerulonephritis. Following the recommendations of The European Vasculitis Study Group (EUVAS) in adult patients, ANCA-associated vasculitis describes the categories of disease severity: localized, early systemic, severe, generalized, refractory. An algorithm for the treatment of ANCA-associated vasculitis, recommended by EULAR (2009) for adult patients and adapted for children of L.A. Plumb et al (2018), which provides for a differentiated approach to the induction of remission in localized, early systemic, severe, generalized, refractory categories of severity and supportive therapy in localized, early systemic, generalized categories, second-line therapy. In most cases of ANCA-associated renal vasculitis in children and adolescents, it is kidney damage that manifests rapidly progressive glomerulonephritis with acute kidney damage, determines the severity and prognosis of outcome in terminal uremia. It seems important and necessary in the treatment protocols of ANCA-associated vasculitis to include a strategy for pre-dialysis and dialysis of rapidly progressive glomerulonephritis with acute kidney damage in children and adolescents.