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ANCA-Associated Vasculitis: An Update.

Published version
Peer-reviewed

Type

Article

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Authors

Fussner, Lynn A 
Brodsky, Sergey 
Meara, Alexa S 
Jayne, David 

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) represents a group of small vessel vasculitides characterized by granulomatous and neutrophilic tissue inflammation, often associated with the production of antibodies that target neutrophil antigens. The two major antigens targeted by ANCAs are leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). AAV can be classified into 3 categories based on patterns of clinical involvement: namely, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA). Clinically, AAV involves many organ systems including the lungs, kidneys, skin, and nervous system. The prognosis of AAV has improved dramatically due to advances in the understanding of its pathogenesis and treatment modalities. This review will highlight some of the recent updates in our understanding of the pathogenesis, clinical manifestations, and treatment options in patients with AAV focusing on kidney involvement.

Description

Keywords

ANCA vasculitis, crescentic glomerulonephritis, review, vasculitis

Journal Title

J Clin Med

Conference Name

Journal ISSN

2077-0383
2077-0383

Volume Title

10

Publisher

MDPI AG