ANCA-Associated Vasculitis: Pathogenesis, Models, and Preclinical Testing.

Abstract:

:Our understanding of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis has developed greatly since the discovery of ANCA, directed against neutrophil components, in 1982. Observations in human disease, and increasingly sophisticated studies in vitro and in rodent models in vivo, have allowed a nuanced understanding of many aspects of the immunopathogenesis of disease, including the significance of ANCA as a diagnostic and monitoring tool as well as a mediator of microvascular injury. The mechanisms of leukocyte recruitment and tissue injury, and the role of T cells increasingly are understood. Unexpected findings, such as the role of complement, also have been uncovered through experimental studies and human observations. This review focusses on the pathogenesis of ANCA-associated vasculitis, highlighting the challenges in finding new, less-toxic treatments and potential therapeutic targets in this disease. The current suite of rodent models is reviewed, and future directions in the study of this complex and fascinating disease are suggested.

journal_name

Semin Nephrol

journal_title

Seminars in nephrology

authors

Hutton HL,Holdsworth SR,Kitching AR

doi

10.1016/j.semnephrol.2017.05.016

subject

Has Abstract

pub_date

2017-09-01 00:00:00

pages

418-435

issue

5

eissn

0270-9295

issn

1558-4488

pii

S0270-9295(17)30055-4

journal_volume

37

pub_type

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