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Immunopathogenesis of ANCA-Associated Vasculitis

Authors
  • Kronbichler, Andreas1
  • Lee, Keum Hwa
  • Denicolò, Sara1
  • Choi, Daeun2
  • Lee, Hyojeong2
  • Ahn, Donghyun2
  • Kim, Kang Hyun2
  • Lee, Ji Han2
  • Kim, HyungTae2
  • Hwang, Minha2
  • Jung, Sun Wook2
  • Lee, Changjun2
  • Lee, Hojune2
  • Sung, Haejune2
  • Lee, Dongkyu2
  • Hwang, Jaehyuk2
  • Kim, Sohee2
  • Hwang, Injae2
  • Kim, Do Young2
  • Kim, Hyung Jun2
  • And 13 more
  • 1 (P.G.)
  • 2 (J.P.)
  • 3 (L.J.)
  • 4 ICREA, Pg. LluisCompanys 23, 08010 Barcelona, Spain
  • 5 Faculty of Medicine, University of Versailles Saint-Quentin-en-Yvelines, 78000 Versailles, France
Type
Published Article
Journal
International Journal of Molecular Sciences
Publisher
MDPI AG
Publication Date
Oct 03, 2020
Volume
21
Issue
19
Identifiers
DOI: 10.3390/ijms21197319
PMID: 33023023
PMCID: PMC7584042
Source
PubMed Central
Keywords
License
Green

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

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