Immunopathogenesis of ANCA-Associated Vasculitis.
Por:
Kronbichler A, Lee KH, Denicolò S, Choi D, Lee H, Ahn D, Kim KH, Lee JH, Kim H, Hwang M, Jung SW, Lee C, Lee H, Sung H, Lee D, Hwang J, Kim S, Hwang I, Kim DY, Kim HJ, Cho G, Cho Y, Kim D, Choi M, Park J, Park J, Tizaoui K, Li H, Smith L, Koyanagi A, Jacob L, Gauckler P and Shin JI
Publicada:
3 oct 2020
Ahead of Print:
3 oct 2020
Resumen:
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.
Filiaciones:
Kronbichler A:
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
Lee KH:
Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Korea
Denicolò S:
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
Choi D:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Lee H:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Ahn D:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim KH:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Lee JH:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim H:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Hwang M:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Jung SW:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Lee C:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Lee H:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Sung H:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Lee D:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Hwang J:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim S:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Hwang I:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim DY:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim HJ:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Cho G:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Cho Y:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Kim D:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Choi M:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Park J:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Park J:
Yonsei University College of Medicine, Yonsei University, Seoul 03722, Korea
Tizaoui K:
Department of Basic Sciences, Division of Histology and Immunology, Faculty of Medicine Tunis, Tunis El Manar University, Tunis 1068, Tunisia
Li H:
University of Florida College of Medicine, University of Florida, Gainesville, FL 32610, USA
Smith L:
The Cambridge Centre for Sport and Exercise Science, Anglia Ruskin University, Cambridge CB1 1PT, UK
Koyanagi A:
Research and Development Unit, Parc Sanitari Sant Joan de Déu, CIBERSAM, 08830 Barcelona, Spain
ICREA, Pg. LluisCompanys 23, 08010 Barcelona, Spain
Jacob L:
Research and Development Unit, Parc Sanitari Sant Joan de Déu, CIBERSAM, 08830 Barcelona, Spain
Faculty of Medicine, University of Versailles Saint-Quentin-en-Yvelines, 78000 Versailles, France
Gauckler P:
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, 6020 Innsbruck, Austria
Shin JI:
Department of Pediatrics, Yonsei University College of Medicine, Seoul 03722, Korea
Green Submitted, Green Published, gold, Green Accepted
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