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2020 international consensus on ANCA testing beyond systemic vasculitis


Autoimmun Rev. 2020 Sep;19(9):102618. doi: 10.1016/j.autrev.2020.102618.Epub 2020 Jul 12.

Sergey Moiseev 1, Jan Willem Cohen Tervaert 2, Yoshihiro Arimura 3, Dimitrios P Bogdanos 4, Elena Csernok 5, Jan Damoiseaux 6, Marc Ferrante 7, Luis Felipe Flores-Suárez 8, Marvin J Fritzler 9, Pietro Invernizzi 10, David Jayne 11, J Charles Jennette 12, Mark A Little 13, Stephen P McAdoo 14, Pavel Novikov 15, Charles D Pusey 14, Antonella Radice 16, Alan D Salama 17, Judith A Savige 18, Mårten Segelmark 19, Yehuda Shoenfeld 20, Renato A Sinico 21, Maria-José Sousa 22, Ulrich Specks 23, Benjamin Terrier 24, Athanasios G Tzioufas 25, Severine Vermeire 7, Ming-Hui Zhao 26, Xavier Bossuyt 27

Author Information

1 Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia. Electronic address: avt420034@yahoo.com.

2 Department of Medicine, Division of Rheumatology, University of Alberta, Edmonton, Canada and Maastricht University, Maastricht, The Netherlands.

3 Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo, Japan.

4 Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, Biopolis, Larissa, Greece.

5 Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany.

6 Central Diagnostic Laboratory, Maastricht University Medical Centre, Maastricht, The Netherlands.

7 University Hospitals Leuven, Department of Gastroenterology and Hepatology, KU Leuven, Leuven, Belgium.

8 Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.

9 Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.

10 Division Gastroenterology and Center for Autoimmune Liver Diseases, University of Milano-Bicocca School of Medicine, Monza, Italy.

11 Department of Medicine, University of Cambridge, Cambridge, UK.

12 Division of Nephropathology, School of Medicine, University of North Carolina, Chapel Hill, NC, USA.

13 Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Dublin, Ireland.

14 Centre for Inflammatory Disease, Department of Medicine, Imperial College London, London, UK.

15 Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia.

16 Microbiology and Virology Institute, ASST Santi Paolo e Carlo, San Carlo Borromeo Hospital, Milan, Italy.

17 UCL Department of Renal Medicine, Royal Free Hospital, London, UK.

18 Department of Medicine, Melbourne Health, University of Melbourne, Melbourne, Australia.

19 Department of Clinical Sciences, Lund University, Department of Nephrology and Rheumatology, Skane University Hospital, Lund, Sweden.

20 Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, Sechenov First Moscow State Medical University, Moscow, Russia.

21 Department of Medicine and Surgery, Università degli Studi di Milano-Bicocca, Monza, Italy.

22 Immunopathology and Autoimmunity Department, Centro de Medicina Laboratorial Germano de Sousa, Lisbon, Portugal.

23 Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

24 Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Paris, France.

25 Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

26 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, Beijing, China.

27 Laboratory Medicine, University Hospitals Leuven, Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.


This document follows up on a 2017 revised international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in granulomatosis with polyangiitis and microscopic polyangiitis and focuses on the clinical and diagnostic value of ANCA detection in patients with connective tissue diseases, idiopathic interstitial pneumonia, autoimmune liver diseases, inflammatory bowel diseases, anti-glomerular basement membrane (GBM) disease, infections, malignancy, and during drug treatment. Current evidence suggests that in certain settings beyond systemic vasculitis, ANCA may have clinical, pathogenic and/or diagnostic relevance. Antigen-specific ANCA targeting proteinase-3 and myeloperoxidase should be tested by solid phase immunoassays in any patient with clinical features suggesting ANCA-associated vasculitis and in all patients with anti-GBM disease, idiopathic interstitial pneumonia, and infective endocarditis associated with nephritis, whereas in patients with other aforementioned disorders routine ANCA testing is not recommended. Among patients with autoimmune liver diseases or inflammatory bowel diseases, ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1 who do not have conventional autoantibodies or in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn's disease. In these cases, ANCA should be tested by indirect immunofluorescence as the target antigens are not yet well characterized. Many questions concerning the optimal use of ANCA testing in patients without ANCA-associated vasculitis remain to be answered.