• A Néel, N Degauque, S Bruneau, C Braudeau, M Bucchia, A Caristan, D De Mornac, V Genin, A Glemain, C Oriot, M Rimbert, S Brouard, R Josien, and M Hamidou.
• Service de médecine interne, CHU de Nantes, Nantes, France; Inserm, centre de recherche en transplantation et immunologie, UMR 1064, université de Nantes, Nantes, France; Centre de référence maladies auto-immunes systémiques Rares, hôpital Cochin, AP-HP, Paris, France. Electronic address: antoine.neel@gmail.com.
• Rev Med Interne. 2022 Feb 1; 43 (2): 89-97.

AbstractAnticytoplasmic neutrophil antibodies (ANCA)-associated vasculitis (AAV) are rare systemic immune-mediated diseases characterized by small vessel necrotizing vasculitis and/or respiratory tract inflammation. Over the last 2 decades, anti-MPO vasculitis mouse model has enlightened the role of ANCA, neutrophils, complement activation, T helper cells (Th1, Th17) and microbial agents. In humans, CD4T cells have been extensively studied, while the dramatic efficacy of rituximab demonstrated the key role of B cells. Many areas of uncertainty remain, such as the driving force of GPA extra-vascular granulomatous inflammation and the relapse risk of anti-PR3 AAV pathogenesis. Animal models eventually led to identify complement activation as a promising therapeutic target. New investigation tools, which permit in depth immune profiling of human blood and tissues, may open a new era for the studying of AAV pathogenesis.Copyright © 2021 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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