Renal Vasculitis in Children View Full Text


Ontology type: schema:Chapter     


Chapter Info

DATE

2016

AUTHORS

Shori Takahashi , Michio Nagata , Hiroshi Saito

ABSTRACT

In this chapter, we focus on antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and glomerulonephritis (GN) as a prototype of “renal vasculitis” in children. Vasculitis is a clinical and pathological “merged” entity that has undergone substantial changes during the past decades; this has created some confusion in understanding it. In particular, several changes in the concept and terminology of vasculitis and the classical clinical entity of rapidly progressive glomerulonephritis (RPGN) have caused confusion in the diagnosis of vasculitis and ANCA-associated GN. The differences in perspectives among nephrologists, rheumatologists, and pathologists have exacerbated the confusion as well. Therefore, we have tried to arrange these perspectives so as to be simple. We will review the recent genome-wide association analysis, which has revealed the genetic difference between granulomatosis with polyangiitis and microscopic polyangiitis, as well as discuss the roles of ANCA itself, human lysosomal membrane protein-2 (hLAMP-2) and neutrophil extracellular traps (NETs) in the pathogenesis of ANCA-associated glomerulonephritis. Recent therapies and the prognosis of ANCA-associated glomerulonephritis are also illustrated. More... »

PAGES

733-757

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-662-52972-0_27

DOI

http://dx.doi.org/10.1007/978-3-662-52972-0_27

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1083891393


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