2020 international consensus on ANCA testing beyond systemic vasculitis

Sergey Moiseev, Jan Willem Cohen Tervaert, Yoshihiro Arimura, Dimitrios P. Bogdanos, Elena Csernok, Jan Damoiseaux, Marc Ferrante, Luis Felipe Flores-Suárez, Marvin J. Fritzler, Pietro Invernizzi, David Jayne, J. Charles Jennette, Mark A. Little, Stephen P. McAdoo, Pavel Novikov, Charles D. Pusey, Antonella Radice, Alan D. Salama, Judith A. Savige, Mårten SegelmarkYehuda Shoenfeld, Renato A. Sinico, Maria José Sousa, Ulrich Specks, Benjamin Terrier, Athanasios G. Tzioufas, Severine Vermeire, Ming Hui Zhao, Xavier Bossuyt

Research output: Contribution to journalReview articlepeer-review

13 Scopus citations


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.

Original languageEnglish (US)
Article number102618
JournalAutoimmunity Reviews
Issue number9
StatePublished - Sep 2020

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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