Immunopathogenesis of ANCA-Associated Vasculitis

Kronbichler, Andreas and Lee, Keum Hwa and Denicolò, Sara and Choi, Daeun and Lee, Hyojeong and Ahn, Donghyun and Kim, Kang Hyun and Lee, Ji Han and Kim, HyungTae and Hwang, Minha and Jung, Sun Wook and Lee, Changjun and Lee, Hojune and Sung, Haejune and Lee, Dongkyu and Hwang, Jaehyuk and Kim, Sohee and Hwang, Injae and Kim, Do Young and Kim, Hyung Jun and Cho, Geonjae and Cho, Yunryoung and Kim, Dongil and Choi, Minje and Park, Junhye and Park, Junseong and Tizaoui, Kalthoum and Li, Han and Smith, Lee and Koyanagi, Ai and Jacob, Louis and Philipp, Gauckler and Shin, Jae Il (2020) Immunopathogenesis of ANCA-Associated Vasculitis. International Journal of Molecular Sciences, 21 (19). p. 7319. ISSN 1422-0067

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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.

Item Type: Journal Article
Keywords: ANCA, Vasculitis, Review, Biomarker, Phenotype, Pathogenesis, Treatment
Faculty: Faculty of Science & Engineering
SWORD Depositor: Symplectic User
Depositing User: Symplectic User
Date Deposited: 01 Oct 2020 09:43
Last Modified: 09 Sep 2021 18:52

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