TY - JOUR TI - 2020 international consensus on ANCA testing beyond systemic vasculitis AU - Moiseev, S. AU - Cohen Tervaert, J.W. AU - Arimura, Y. AU - Bogdanos, D.P. AU - Csernok, E. AU - Damoiseaux, J. AU - Ferrante, M. AU - Flores-Suárez, L.F. AU - Fritzler, M.J. AU - Invernizzi, P. AU - Jayne, D. AU - Jennette, J.C. AU - Little, M.A. AU - McAdoo, S.P. AU - Novikov, P. AU - Pusey, C.D. AU - Radice, A. AU - Salama, A.D. AU - Savige, J.A. AU - Segelmark, M. AU - Shoenfeld, Y. AU - Sinico, R.A. AU - Sousa, M.-J. AU - Specks, U. AU - Terrier, B. AU - Tzioufas, A.G. AU - Vermeire, S. AU - Zhao, M.-H. AU - Bossuyt, X. JO - AUTOIMMUNITY REVIEWS PY - 2020 VL - 19 TODO - 9 SP - null PB - Elsevier B.V. SN - 1568-9972 TODO - 10.1016/j.autrev.2020.102618 TODO - neutrophil cytoplasmic antibody; myeloblastin; neutrophil cytoplasmic antibody; peroxidase, ANCA associated vasculitis; autoimmune liver disease; consensus; drug induced disease; glomerulonephritis; human; infection; inflammatory bowel disease; interstitial lung disease; malignant neoplasm; medical terminology; nonhuman; Review; rheumatoid arthritis; Sjoegren syndrome; systemic lupus erythematosus; systemic sclerosis; systemic vasculitis; ANCA associated vasculitis; autoimmune hepatitis; immunology; Wegener granulomatosis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Consensus; Granulomatosis with Polyangiitis; Hepatitis, Autoimmune; Humans; Myeloblastin; Peroxidase TODO - 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. © 2020 Elsevier B.V. ER -