TY - JOUR
T1 - International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis
AU - Moiseev, Sergey
AU - Bossuyt, Xavier
AU - Arimura, Yoshihiro
AU - Blockmans, Daniel
AU - Csernok, Elena
AU - Damoiseaux, Jan
AU - Emmi, Giacomo
AU - Flores-Suarez, Luis Felipe
AU - Hellmich, Bernhard
AU - Jayne, David
AU - Jennette, J. Charles
AU - Little, Mark A.
AU - Mohammad, Aladdin J.
AU - Moosig, Frank
AU - Novikov, Pavel
AU - Pagnoux, Christian
AU - Radice, Antonella
AU - Sada, Ken-ei
AU - Segelmark, Marten
AU - Shoenfeld, Yehuda
AU - Sinico, Renato A.
AU - Specks, Ulrich
AU - Terrier, Benjamin
AU - Tzioufas, Athanasios G.
AU - Vaglio, Augusto
AU - Zhao, Ming-Hui
AU - Tervaert, Jan Willem Cohen
AU - European EGPA Study Group
PY - 2020/11/15
Y1 - 2020/11/15
N2 - 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.
AB - 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.
KW - eosinophilic granulomatosis with polyangiitis
KW - ANCA
KW - vasculitis
KW - consensus
KW - CHURG-STRAUSS-SYNDROME
KW - TERM-FOLLOW-UP
KW - CARDIAC INVOLVEMENT
KW - SYSTEMIC VASCULITIS
KW - RITUXIMAB
KW - EGPA
KW - MYELOPEROXIDASE
KW - AUTOANTIBODIES
KW - CYCLOPHOSPHAMIDE
U2 - 10.1164/rccm.202005-1628SO
DO - 10.1164/rccm.202005-1628SO
M3 - Article
C2 - 32584187
VL - 202
SP - 1360
EP - 1372
JO - American Journal of Respiratory and Critical Care Medicine
JF - American Journal of Respiratory and Critical Care Medicine
SN - 1073-449X
IS - 10
ER -