2020 international consensus on ANCA testing beyond systemic vasculitis

Research output: Contribution to journalReview article


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.


  • 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
  • Yehuda Shoenfeld
  • Renato A. Sinico
  • Maria José Sousa
  • Ulrich Specks
  • Benjamin Terrier
  • Athanasios G. Tzioufas
  • Severine Vermeire
  • Ming Hui Zhao
  • Xavier Bossuyt
External organisations
  • I.M. Sechenov First Moscow State Medical University
  • University of Alberta
  • University of Thessaly
  • University of Tübingen
  • University Hospitals Leuven
  • Instituto Nacional de Enfermedades Respiratorias
  • University of Calgary
  • University of Milano-Bicocca
  • University of Cambridge
  • University of North Carolina
  • Imperial College London
  • San Carlo Borromeo Hospital, Milan
  • Royal Free Hospital
  • University of Melbourne
  • Skåne University Hospital
  • Tel-Aviv University
  • Centro de Medicina Laboratorial Germano de Sousa
  • Mayo Clinic
  • Cochin Hospital
  • National and Kapodistrian University of Athens
  • Peking University First Hospital
  • Kyorin University
  • Medius Klinik Kirchheim
  • Maastricht University Medical Centre
  • Catholic University of Leuven
  • Trinity College Dublin
  • Sheba Medical Center
  • Peking University
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Urology and Nephrology
Original languageEnglish
Article number102618
JournalAutoimmunity Reviews
Publication statusE-pub ahead of print - 2020 Jul 12
Publication categoryResearch