TY - JOUR
T1 - Immunopathogenesis of ANCA-associated vasculitis
AU - Kronbichler, Andreas
AU - Lee, Keum Hwa
AU - Denicolò, Sara
AU - Choi, Daeun
AU - Lee, Hyojeong
AU - Ahn, Donghyun
AU - Kim, Kang Hyun
AU - Lee, Ji Han
AU - Kim, Hyungtae
AU - Hwang, Minha
AU - Jung, Sun Wook
AU - Lee, Changjun
AU - Lee, Hojune
AU - Sung, Haejune
AU - Lee, Dongkyu
AU - Hwang, Jaehyuk
AU - Kim, Sohee
AU - Hwang, Injae
AU - Kim, Do Young
AU - Kim, Hyung Jun
AU - Cho, Geonjae
AU - Cho, Yunryoung
AU - Kim, Dongil
AU - Choi, Minje
AU - Park, Junhye
AU - Park, Junseong
AU - Tizaoui, Kalthoum
AU - Li, Han
AU - Smith, Lee
AU - Koyanagi, Ai
AU - Jacob, Louis
AU - Gauckler, Philipp
AU - Shin, Jae Il
N1 - Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2020/10/1
Y1 - 2020/10/1
N2 - 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.
AB - 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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U2 - 10.3390/ijms21197319
DO - 10.3390/ijms21197319
M3 - Review article
C2 - 33023023
AN - SCOPUS:85091998712
SN - 1661-6596
VL - 21
SP - 1
EP - 27
JO - International Journal of Molecular Sciences
JF - International Journal of Molecular Sciences
IS - 19
M1 - 7319
ER -