Author:

Keywords:

Science & Technology, Life Sciences & Biomedicine, Critical Care Medicine, Respiratory System, General & Internal Medicine, eosinophilic granulomatosis with polyangiitis, ANCA, vasculitis, consensus, CHURG-STRAUSS-SYNDROME, TERM-FOLLOW-UP, CARDIAC INVOLVEMENT, SYSTEMIC VASCULITIS, RITUXIMAB, EGPA, MYELOPEROXIDASE, AUTOANTIBODIES, CYCLOPHOSPHAMIDE, European EGPA Study Group, 11 Medical and Health Sciences, 3201 Cardiovascular medicine and haematology, 3202 Clinical sciences

Abstract:

An 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.