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International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with
Sergey Moiseev1, Xavier Bossuyt2, Yoshihiro Arimura3
1Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia.
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.
Insights
Anti-neutrophil cytoplasm antibodies (ANCA) testing is recommended for diagnosing eosinophilic granulomatosis with polyangiitis (EGPA). Myeloperoxidase (MPO)-ANCA aids diagnosis but doesn't guide EGPA subclassification or treatment.
Area of Science:
- Rheumatology
- Immunology
- Pulmonology
Background:
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis.
- Anti-neutrophil cytoplasm antibodies (ANCA), particularly those against myeloperoxidase (MPO), are associated with EGPA.
- International consensus is needed for ANCA testing in EGPA diagnosis and management.
Purpose of the Study:
- To present an international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA).
- To define the role of MPO-ANCA in the diagnostic work-up of EGPA.
- To clarify the utility of ANCA testing in EGPA subclassification and treatment monitoring.
Main Methods:
- Review of existing literature and expert opinion.
- Development of consensus recommendations for MPO-ANCA testing in suspected EGPA.
- Analysis of the diagnostic, prognostic, and therapeutic implications of MPO-ANCA in EGPA.
Main Results:
- Myeloperoxidase-specific ANCA (MPO-ANCA) is detected in 30-35% of EGPA patients.
- MPO-ANCA testing is recommended for patients with eosinophilic asthma and suggestive clinical features.
- MPO-ANCA aids EGPA diagnosis and is associated with more frequent vasculitic manifestations but does not guide subclassification or treatment decisions.
Conclusions:
- MPO-ANCA testing should be performed using antigen-specific immunoassays in patients with suspected EGPA.
- While MPO-ANCA contributes to EGPA diagnosis, it is neither sensitive nor specific enough for subclassification.
- Monitoring ANCA levels in EGPA is only useful if MPO-ANCA was positive at disease onset; current ANCA status does not guide treatment choices.
