International Journal of Molecular Sciences (Oct 2020)
Immunopathogenesis of ANCA-Associated Vasculitis
- Andreas Kronbichler,
- Keum Hwa Lee,
- Sara Denicolo,
- Daeun Choi,
- Hyojeong Lee,
- Donghyun Ahn,
- Kang Hyun Kim,
- Ji Han Lee,
- HyungTae Kim,
- Minha Hwang,
- Sun Wook Jung,
- Changjun Lee,
- Hojune Lee,
- Haejune Sung,
- Dongkyu Lee,
- Jaehyuk Hwang,
- Sohee Kim,
- Injae Hwang,
- Do Young Kim,
- Hyung Jun Kim,
- Geonjae Cho,
- Yunryoung Cho,
- Dongil Kim,
- Minje Choi,
- Junhye Park,
- Junseong Park,
- Kalthoum Tizaoui,
- Han Li,
- Lee Smith,
- Ai Koyanagi,
- Louis Jacob,
- Philipp Gauckler,
- Jae Il Shin
Affiliations
- Andreas Kronbichler
- Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
- Keum Hwa Lee
- Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Korea
- Sara Denicolo
- Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
- Daeun Choi
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Hyojeong Lee
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Donghyun Ahn
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Kang Hyun Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Ji Han Lee
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- HyungTae Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Minha Hwang
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Sun Wook Jung
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Changjun Lee
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Hojune Lee
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Haejune Sung
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Dongkyu Lee
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Jaehyuk Hwang
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Sohee Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Injae Hwang
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Do Young Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Hyung Jun Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Geonjae Cho
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Yunryoung Cho
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Dongil Kim
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Minje Choi
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Junhye Park
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Junseong Park
- Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
- Kalthoum Tizaoui
- Department of Basic Sciences, Division of Histology and Immunology, Faculty of Medicine Tunis, Tunis El Manar University, Tunis 1068, Tunisia
- Han Li
- University of Florida College of Medicine, University of Florida, Gainesville, FL 32610, USA
- Lee Smith
- The Cambridge Centre for Sport and Exercise Science, Anglia Ruskin University, Cambridge CB1 1PT, UK
- Ai Koyanagi
- Research and Development Unit, Parc Sanitari Sant Joan de Déu, CIBERSAM, 08830 Barcelona, Spain
- Louis Jacob
- Research and Development Unit, Parc Sanitari Sant Joan de Déu, CIBERSAM, 08830 Barcelona, Spain
- Philipp Gauckler
- Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
- Jae Il Shin
- Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Korea
- DOI
- https://doi.org/10.3390/ijms21197319
- Journal volume & issue
-
Vol. 21,
no. 19
p. 7319
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.
Keywords