• Hugues Allard-Chamard and Patrick Liang.
• Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, USA; Division of Rheumatology, Faculty of Medicine and Health Sciences, Université de Sherbrooke; Centre de Recherche Clinique du Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Québec, Canada; Division of Rheumatology, Centre intégré universitaire de santé et de service sociaux de l'Estrie - Centre hospitalier universitaire de Sherbrooke (CIUSSS de l'Estrie-CHUS), 3001, 12th Avenue North, Room 3853, Sherbrooke, Québec J1H 5N4, Canada. Electronic address: hugues.allard-chamard@USherbrooke.ca.
• Clin. Lab. Med. 2019 Dec 1; 39 (4): 539-552.

AbstractThe discovery of antineutrophil cytoplasmic antibodies (ANCA) helped establish ANCA-associated vasculitis as a separate and well-defined clinical entity. Its progressive incorporation into the clinical diagnosis algorithms has made ANCA testing a cornerstone immunoassay embedded in the management of ANCA-associated vasculitis. After its description by indirect immunofluorescence, proteinase-3 and myeloperoxidase were identified as principal ANCA targets. ANCA, and proteinase-3 and myeloperoxidase immunoassessment, have undergone iterative rounds of improvement in sensitivity and specificity. This article traces landmarks in the development of ANCA tests, describes common pitfalls arising during ANCA interpretation, and discusses new technologies to improve the future of ANCA testing.Copyright © 2019 Elsevier Inc. All rights reserved.

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