• Sergey Moiseev, Xavier Bossuyt, Yoshihiro Arimura, Daniel Blockmans, Elena Csernok, Jan Damoiseaux, Giacomo Emmi, Luis Felipe Flores-Suárez, Bernhard Hellmich, David Jayne, J Charles Jennette, Mark A Little, Aladdin J Mohammad, Frank Moosig, Pavel Novikov, Christian Pagnoux, Antonella Radice, Ken-Ei Sada, Mårten Segelmark, Yehuda Shoenfeld, Renato A Sinico, Ulrich Specks, Benjamin Terrier, Athanasios G Tzioufas, Augusto Vaglio, Ming-Hui Zhao, Cohen TervaertJan WillemJW0000-0001-7407-6364University of Alberta, Medicine, Edmonton, Alberta, Canada.Maastricht University, MHeNS, Maastricht, Netherlands., and European EGPA Study Group.
• Sechenov First Moscow State Medical University, Tareev Clinic of Internal Diseases, Moscow, Russian Federation; avt420034@yahoo.com.
• Am. J. Respir. Crit. Care Med. 2020 Jun 25.

AbstractAn international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms, purpura, polyneuropathy, unexplained heart, gastrointestinal or kidney disease, and/or pulmonary infiltrates or hemorrhage. A positive MPO-ANCA result contributes to the diagnostic work‑up for EGPA. Patients with MPO-ANCA associated EGPA have more frequently vasculitis features, such as glomerulonephritis, neuropathy, and skin manifestations than patients with ANCA negative EGPA. However, the presence of MPO-ANCA is neither sensitive nor specific enough to identify whether a patient should be subclassified as having "vasculitic" or "eosinophilic" EGPA. At present, ANCA status cannot guide treatment decisions, that is, whether cyclophosphamide, rituximab or mepolizumab should be added to conventional glucocorticoid treatment. In EGPA, monitoring of ANCA is only useful when MPO-ANCA was tested positive at disease onset.

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