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Nat Rev Rheumatol. 2017 Nov;13(11):683-692. doi: 10.1038/nrrheum.2017.140. Epub 2017 Sep 14.

Position paper: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis.

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Department of Microbiology and Immunology, University of Leuven and Department of Laboratory Medicine, University Hospitals Leuven, Leuven, Herestraat 49, 3000 Leuven, Belgium.
Maastricht University, Universiteitssingel 40, 6229 Maastricht, Netherlands.
Kichijoji Asahi Hospital, 11-30-12 Kichijoji Honcho, Musashino, Tokyo 181-8611, Japan.
Clinical Department of General Internal Medicine, Research Department of Microbiology and Immunology and Laboratory of Clinical Infectious and Inflammatory Disorders, University Hospitals Leuven, Leuven, Herestraat 49, 3000 Leuven, Belgium.
Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Calzada de Tlalpan 4502, Colonia Sección XVI, CP 14080, Mexico City, Mexico.
National Referral Centre for Necrotizing Vasculitides and Systemic Sclerosis, Université Paris Descartes, Hôpital Cochin, L'Assistance Publique-Hôpitaux de Paris, 27 Rue du Faubourg Saint-Jacques, Paris 75014, France.
Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Centre Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Eugenstrasse 3, 73230 Kirchheim unter Teck, Germany.
Department of Medicine, University of Cambridge, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 0SP, UK.
Department of Pathology and Laboratory Medicine, University of North Carolina, 160 Medical Drive, Chapel Hill, North Carolina 27599, USA.
Department of Rheumatology and Clinical Immunology, AA21, University Medical Centre Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, Netherlands.
Clinic of Nephrology, Internal and Occupational Diseases, Sechenov First Moscow State Medical University, Rossolimo, 11/5, Moscow 119435, Russia.
Microbiology and Virology Institute, ASST Santi Paolo e Carlo, San Carlo Borromeo Hospital, Via Pio II 3, 20153 Milan, Italy.
Department of Medicine, Melbourne Health, The University of Melbourne, Grattan Street, Parkville, Melbourne VIC 3050, Australia.
Department of Medicine and Surgery, Università degli Studi di Milano-Bicocca, Via Cadore, 48, 20900 Monza MB, Italy.
Division of Pulmonary & Critical Medicine, Mayo Clinic, Rochester, 200 First Street, Rochester, Minnesota 55905, USA.
Department of Internal Medicine, Section Nephrology and Immunology, Maastricht University Medical Centre, P. Debyelaan 25, 6229 HX Maastricht, Netherlands.
Renal Division, Peking University First Hospital; Key Laboratory of Renal Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China; Peking-Tsinghua Centre for Life Sciences; 8 Xishiku Street, Xichengqu, Beijing Shi, China.
Department of Autoimmunology and Biomarkers, Statens Seruminstitut, Artillerivej 5, 2300 Copenhagen S, Denmark.
Central Diagnostic Laboratory, Maastricht University Medical Centre, P. Debyelaan 25, 6229 HX Maastricht, Netherlands.


Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications. As dependable immunoassays for PR3-ANCAs and MPO-ANCAs have become broadly available, there is increasing international agreement that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis. The present Consensus Statement proposes that high-quality immunoassays can be used as the primary screening method for patients suspected of having the ANCA-associated vaculitides GPA and MPA without the categorical need for IIF, and presents and discusses evidence to support this recommendation.

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