A case of rapidly progressive glomerulonephritis in a 17-year-old patient associated with antibodies against the cytoplasm of neutrophils (ANCA) vasculitis - ANCA-associated vasculitis is associated with antibodies to proteinase-3 and morphological picture extracapillar glomerulonephritis with sclerotic lesion of up to 80% of the glomeruli. The peculiarity of the case is the presence of morphologically confirmed when alloimmune rapidly progressive glomerulonephritis type III a pronounced glow-focal granular nature of immunoglobulin classes G and M on the basement membrane of capillaries. The appointment of immunosuppressive therapy led to a decrease in systemic manifestations of vasculitis, but there was a rapid increase in terminal renal failure, which required substitution therapy with hemodialysis. The possible mechanisms of the rapid-training course of the disease in the observed patient, prospects for kidney transplantation are discussed.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are a group of systemic autoimmune disorders characterized by necrotizing inflammation of medium-to-small vessels, a relative paucity of immune deposits, and an association with detectable circulating ANCAs. AAVs include granulomatosis with polyangiitis (renamed from Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Until recently, AAVs have not been viewed as complement-mediated disorders. However, recent findings predominantly from animal studies demonstrated a crucial role of the complement system in the pathogenesis of AAVs. Complement activation or defects in its regulation have been described in an increasing number of acquired or genetically driven forms of thrombotic microangiopathy. Coinciding with this expanding spectrum of complement-mediated diseases, the question arises as to which AAV patients might benefit from a complement-targeted therapy. Therapies directed against the complement system point to the necessity of a genetic workup of genes of complement components and regulators in patients with AAV. Genetic testing together with pluripotent stem cells and bioinformatics tools may broaden our approach to the treatment of patients with aggressive forms of AAV.
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