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- W3140623185 endingPage "733" @default.
- W3140623185 startingPage "719" @default.
- W3140623185 abstract "IgA vasculitis with nephritis (IgAVN) shares many pathogenetic features with IgA nephropathy (IgAN). The purpose of this review is to describe our current understanding of the pathogenesis of pediatric IgAVN, particularly as it relates to the four-hit hypothesis for IgAN. These individual steps, i.e., hits, in the pathogenesis of IgAN are (1) elevated production of IgA1 glycoforms with some O-glycans deficient in galactose (galactose-deficient IgA1; Gd-IgA1), (2) generation of circulating IgG autoantibodies specific for Gd-IgA1, (3) formation of pathogenic circulating Gd-IgA1-containing immune complexes, and (4) kidney deposition of the Gd-IgA1-IgG immune complexes from the circulation and induction of glomerular injury. Evidence supporting the four-hit hypothesis in the pathogenesis of pediatric IgAVN is detailed. The genetics, pediatric outcomes, and kidney histopathologic features and the impact of these findings on future treatment and potential biomarkers are discussed. In summary, the evidence points to the critical roles of Gd-IgA1-IgG immune complexes and complement activation in the pathogenesis of IgAVN. Future studies are needed to characterize the features of the immune and autoimmune responses that enable progression of IgA vasculitis to IgAVN." @default.
- W3140623185 created "2021-04-13" @default.
- W3140623185 creator A5000517864 @default.
- W3140623185 creator A5011125409 @default.
- W3140623185 creator A5024741642 @default.
- W3140623185 creator A5031087263 @default.
- W3140623185 creator A5050736159 @default.
- W3140623185 creator A5080220401 @default.
- W3140623185 creator A5091322640 @default.
- W3140623185 date "2021-04-05" @default.
- W3140623185 modified "2023-10-14" @default.
- W3140623185 title "IgA vasculitis with nephritis: update of pathogenesis with clinical implications" @default.
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