International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis
Sergey Moiseev, Xavier Bossuyt, Yoshihiro Arimura, Daniël 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 Alberto Sinico, Ulrich Specks, Benjamin Terrier, Athanasios G. Tzioufas, Augusto Vaglio, Ming‐Hui Zhao, Jan Willem Cohen Tervaert
Abstract
An international consensus on antineutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of patients with EGPA. 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 workup for EGPA. Patients with MPO-ANCA-associated EGPA have vasculitis features, such as glomerulonephritis, neuropathy, and skin manifestations, more frequently 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.