Yunryoung Cho, Minha Hwang, Junhye Park, Hyo Jeong Lee, Kang Hyun Kim, Lee Smith, Ji Han Lee, Jae Il Shin, Han Li, Sun Wook Jung, Junseong Park, Hyungjun Kim, Do Young Kim, Andreas Kronbichler, Haejune Sung, Sara Denicolò, Hojune Lee, Geonjae Cho, Dong-Il Kim, Daeun Choi, Philipp Gauckler, Keum Hwa Lee, Dongkyu Lee, Hyung Tae Kim, Jaehyuk Hwang, Ai Koyanagi, Injae Hwang, Sohee Kim, Changjun Lee, Min Je Choi, Louis Jacob, Kalthoum Tizaoui, Donghyun Ahn, Innsbruck Medical University [Austria] (IMU), Yonsei University, Faculté de médecine - Faculty of Medicine [Sfax, Tunisie] (FMS), Université de Sfax - University of Sfax, University of Florida [Gainesville] (UF), Anglia Ruskin University (ARU), Centro de Investigación Biomédica en Red Salud Mental [Madrid] (CIBER-SAM), ICREA Infection Biology Laboratory (Department of Experimental and Health Sciences), Universitat Pompeu Fabra [Barcelona] (UPF), Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), and Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)
International audience; 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.